18: Plastics, Skin, and Soft Tissue Flashcards

Question

Soft tissue sarcoma chemotherapy is based with which drug?

Answer 1

Doxorubicin [UpToDate: The only single agents that are consistently associated with response rates of more than 20 percent in metastatic STS are doxorubicin, epirubicin, and ifosfamide. Even for these agents, the range of objective activity between various small (and even larger) trials is impressive, demonstrating the variability in disease sensitivity and the fact that any given STS patient population could serve as an important confounding variable for interpretation of drug efficacy. The sensitivity of STS to systemic chemotherapy was first demonstrated with single agent doxorubicin in the early 1970s, and subsequent studies suggested a dose-response relationship. The threshold dose for optimal activity appears be ≥60 mg/m2 per cycle, usually administered once every three weeks, with lower doses associated with inferior antitumor activity. It is difficult to demonstrate a clinically meaningful dose-response relationship with single agent doxorubicin at doses beyond 75 mg/m2 per cycle, which has become the standard dose. Even in modern multi-institutional series using 70 to 80 mg/m2 per cycle, there is significant variability in reported response rates, which range from 10 to 25 percent. The vast majority are partial, rather than complete, responses. Doxorubicin is associated with reversible myelosuppression, mucositis, alopecia, nausea and vomiting, and both acute and chronic cardiotoxicity. This drug has a relatively narrow therapeutic index; even small variations of dose beyond 75 mg/m2 can drastically worsen patient tolerance, and even 75 mg/m2 is considered by many patients to be significantly more toxic than 60 mg/m2. Infusional, rather than bolus, administration reduces the likelihood of cardiotoxicity. Other methods to diminish cardiotoxicity include the concomitant use of the cardioprotectant dexrazoxane or the use of liposome-encapsulated doxorubicin.]

Answer 2

Incisional biopsy (punch biopsy) [If pathology comes back as melanoma then need resection with margins.]

Answer 3

Basal cell carcinoma [4 times more common than squamous cell skin cancer.] [UpToDate: Estimates of the incidence of BCC are imprecise since there is no cancer registry that collects data on BCC. The American Cancer society estimates that more than two million nonmelanoma skin cancers were treated in the United States in 2006, of which the majority would have been BCC. A population-based study with several methodologic limitations estimated a higher number of lesions, reporting that approximately 3.5 million nonmelanoma skin cancers were treated in the United States during the same year.]

Answer 4

Actinic keratoses [Seborrheic keratoses are not premalignant and often occur on the torso in elderly populations. Actinic keratoses are premalignant and occur in sun-damaged areas. Actinic keratoses need an excisional biopsy if suspicious.] [UpToDate: The likelihood of progression of an individual AK to SCC is low. Estimates of annual rates of transformation have ranged from 0.03 to 20 percent. The following data come from two of the largest studies: 1. An Australian study that evaluated 1689 adults over the age of 40 who had more than 20,000 AKs found the risk for transformation of an individual AK to SCC within one year to be less than 0.1 percent. 2. Analysis of data from a cohort of patients in a United States trial designed to investigate the use of topical tretinoin for skin cancer prevention in patients with at least two prior keratinocyte carcinomas revealed a rate of transformation to invasive or in situ SCC of 0.6 percent in 6015 AKs followed for one year and a rate of 2.6 percent in 1480 lesions followed for four years. Progression of AKs to basal cell carcinoma (BCC) occurred in 0.5 percent of AKs within one year and in 1.6 percent within four years. However, it is unclear whether this finding was related the misdiagnosis of early BCCs as AKs or was true disease progression. Although few AKs progress to SCC, data from these studies suggest that approximately 60 percent of cutaneous SCCs arise from preexisting AKs. This observation supports a close relationship between these lesions.]

Answer 5

MRI to rule out vascular, neuro, or bone invasion [UpToDate: Histologic examination of a soft tissue mass is essential for diagnosis and treatment planning. There are several methods for obtaining a biopsy, but ideally, the biopsy should be performed after an MRI has been obtained as post-procedural edema may make the MRI difficult to interpret.]

Answer 6

Excisional biopsy (tru-cut core needle biopsy) [If pathology comes back as melanoma then need resection with margins.]

Answer 7

Malignant fibrous histiosarcoma (also known as pleomorphic undifferentiated sarcoma) [#2 is liposarcoma]

Answer 8

Neurofibromatosis (Von Recklinghausen's disease)

Answer 9

Periumbilical perforators [UpToDate: The skin and subcutaneous fat of the lower abdomen receive their blood supply from the underlying muscle via perforating vessels from the superior and inferior epigastric arteries, which arborize through the rectus abdominus muscle.]

Answer 10

Involves bony cortex and muscle [Treatment: Myocutaneous flap.]

Answer 11

Von Recklinghausen's disease (Neurofibromatosis type 1)

Answer 12

Eccrine sweat glands [Responsible for thermal regulation, usually hypotonic.]

Answer 13

Yes [Clinically positive nodes are usually nontender, round, hard, and 1-2 cm in size] [UpToDate: Lymphatic mapping with sentinel lymph node biopsy (SLNB) is the standard clinicopathologic approach to evaluate regional lymph nodes in patients without clinical evidence of lymph node involvement. For patients with clinically negative regional lymph nodes, the decision whether or not to perform an SLNB is based upon the likelihood of regional lymph node involvement. For patients with a melanoma 0.75 mm thick or greater, we recommend performing an SLNB. This criterion includes patients with intermediate and thick primary lesions, as well as those with thin melanomas 0.75 to 1.00 mm thick. For patients with a melanoma less than 0.75 mm thick and one or more factors placing the patient at increased risk for nodal involvement (ulceration of the primary tumor, a mitotic rate ≥ 1/mm2, or lymphovascular invasion), we favor performing an SLNB. However, the absolute risk of a positive sentinel lymph node is small in this group, and a detailed discussion with the patient is needed to discuss the potential risks and benefits. SLNB is not indicated for patients with a melanoma \<0.75 mm and no other factors placing the patient at increased risk for nodal involvement. For patients with a positive SLNB, we suggest completion lymph node dissection (Grade 2B). In patients with a positive SLNB, adjuvant therapy with interferon alfa should be limited to patients with a life expectancy of ten years in the absence of serious comorbidity. Participation in a clinical trial is also an option, especially for patients not considered candidates for or uninterested in receiving interferon alfa. For patients who present with clinically apparent regional lymph node involvement that is confirmed cytologically (fine needle aspirate) or histologically, we recommend therapeutic regional lymphadenectomy (Grade 1B). Partial dissection or lymph node sampling is not considered an acceptable alternative because of the propensity of melanoma to spread microscopically to other nodes in the basin.]

Answer 14

Structural proteins such as collagen

Answer 15

Superficial parotidectomy [20% metastasis rate to parotid.]

Answer 16

Superior epigastric vessels [Complications include flap necrosis, ventral hernia, bleeding, infection, abdominal wall weakness.] [UpToDate: One of the most commonly used autologous tissue reconstructions is the TRAM (transverse rectus abdominus myocutaneous) flap. The flap is comprised of an ellipse of lower abdominal skin, subcutaneous fat, and muscle. The skin and subcutaneous fat of the lower abdomen receive their blood supply from the underlying muscle via perforating vessels from the superior and inferior epigastric arteries, which arborize through the rectus abdominus muscle. The pedicled TRAM flap is based on the superior epigastric artery and vein, and is rotated into the breast pocket with the superior portion of the muscle (and blood supply to the flap) still attached to the costal margin. TRAM flaps use a transversely oriented skin island from the lower abdomen. This design incorporates an abdominoplasty ("tummy tuck") as part of the donor site closure. Using a single rectus muscle, up to 75 percent of the infraumbilical skin and fat can be harvested for the pedicled TRAM flap.]

Answer 17

Morpheaform type [It has collagenase production.] [UpToDate: Morpheaform or sclerosing BCCs constitute 5 to 10 percent of BCCs. These lesions are typically smooth, flesh-colored, or very lightly erythematous papules or plaques that are frequently atrophic; they usually have a firm or indurated quality with ill-defined borders. Some authors group morpheaform, infiltrative, and micronodular as "aggressive-growth" BCC, because they behave similarly. Infiltrative and micronodular subtypes are less common than the morpheaform BCC.]

Answer 18

Alveolar subtype [Embryonal subtype is the most common.] [UpToDate: In the past, the presence of any alveolar pattern was sufficient to categorize a tumor as an alveolar RMS. However, in most recent protocols the Soft Tissue Sarcoma committee of the Children's Oncology Group has recommended that the lesion must have a predominant (\>50 percent) alveolar component (or bear the characteristic translocations t(1;13) or t(2;13)) to be subtyped as alveolar. The typical appearance is that of fibrovascular septae that are lined with densely packed ovoid to round tumor cells and separated by pseudo-alveolar spaces, which vaguely resemble pulmonary alveoli. Frequently, the “loosely adherent” rhabdomyoblasts are shed into these pseudo-alveolar spaces. Less well-differentiated alveolar RMS may feature only a suggestion of fine fissuring or microalveoli; molecular techniques may aid in the diagnosis of such cases.]

Answer 19

* Oral mucosa * Pharyngeal mucosa [Symptoms in these regions include bleeding and dysphagia. Kaposi's sarcoma is a vascular sarcoma.] [UpToDate: During the course of the disease (rarely initially), mucous membranes of mouth and gastrointestinal (GI) tract and regional lymph nodes may be affected. GI tract involvement is usually asymptomatic, but bleeding, diarrhea, protein-losing enteropathy, intussusception, and perforation have been reported. In general, GI tract/oral mucosal involvement is less common than with AIDS-related KS, affecting ≤10 percent of patients. However, in one report, an extraordinary 82 percent of Greek patients (71 of 87) with biopsy-proven CKS who were investigated with upper gastrointestinal endoscopy had GI lesions; all 71 had stomach lesions, 19 had esophageal lesions, eight had lesions of the proximal duodenum, and two had both esophageal and duodenal lesions. Although this finding could be interpreted as suggesting the need for screening endoscopy in patients with newly diagnosed CKS, it also supports the view that the presence of asymptomatic GI involvement probably has little effect on prognosis. In practice, routine endoscopy is not performed in people diagnosed with CKS who do not have symptoms referable to the GI tract.]

Answer 20

Thoracic sympathectomy if refractory to variety of antiperspirants [Symptoms include increased sweating that is especially noticeable in the palms.] [UpToDate: When choosing treatments for primary focal hyperhidrosis, the patient's goals in therapy should be understood and the side effects associated with each therapy should be carefully discussed. Conservative measures should be tried before progressing to more invasive treatments. The severity and location of hyperhidrosis helps guide the choice of therapies. Patients with axillary, palmar, or plantar hyperhidrosis should initially be treated with topical antiperspirants such as aluminum chloride hexahydrate in alcohol. Skin irritation is a common side effect. Iontophoresis is an alternative initial treatment for patients with palmar or plantar hyperhidrosis. Botulinum toxin injection is an effective second-line treatment for axillary hyperhidrosis and microwave thermolysis is a less-widely available alternative. Palmar and plantar hyperhidrosis can also be treated with botulinum toxin, but the injections are quite painful on the hands and feet, and a significant percentage of patients develop temporary local muscle weakness. Patients with axillary hyperhidrosis who cannot be managed effectively with first- or second-line treatments may benefit from suction curettage, a local surgical intervention. If this is not feasible or effective, our preferred next therapy is systemic medication. Endoscopic thoracic sympathectomy (ETS) is an additional option. The selection of these treatments should follow careful consideration of the associated risks and side effects. Patients with palmar hyperhidrosis who cannot be managed effectively with antiperspirants, iontophoresis, or botulinum toxin are candidates for systemic medications or sympathectomy. First-line therapies for craniofacial hyperhidrosis include topical antiperspirants. Patients who cannot be managed with topical therapy may benefit from botulinum toxin injections or oral medication.]

Answer 21

_\>_ 1 mm deep [UpToDate: SLNB is the standard approach for the management of patients with melanoma in whom there is a substantial risk of regional node metastasis. The likelihood of detecting metastatic deposits in a SLNB increases with the thickness of the primary lesion, providing a rationale for deciding which patients may benefit from this procedure. The generally accepted approach focuses on tumor thickness and related risk factors: SLNB is indicated for melanomas ≥1 mm thick. For lesions 1.01 to 2.0, 2.01 to 4.0, and \>4 mm, the risk of regional lymph node metastasis is approximately 12, 28, and 44 percent, respectively. For thin melanomas (ie, \<1 mm thick), the overall risk of regional node metastases in patients undergoing SLNB is estimated to be about 5 percent. A retrospective review from the Sentinel Lymph Node Working Group of 1250 patients with thin melanoma who underwent SLNB provides the most extensive data in this group. Breslow thickness ≥0.75 mm, Clark level ≥IV, and ulceration were associated with lymph node metastases in 6.3, 7.0, and 11.6 percent of cases, respectively, and these factors were all statistically significant on multivariate analysis. In contrast, melanomas \<0.75 mm thick had positive SLNBs less than 5 percent of the time regardless of the Clark level or the presence of ulceration. The low risk of a positive SLNB in patients with a primary lesion \<0.75 mm and without any other risk factors has also been seen in earlier studies. **In summary, for patients with a melanoma 0.75 mm thick or greater, we recommend performing an SLNB.**]

Answer 22

Type-I collagen [Composes 70% of dermis. Gives tensile strength.]

Answer 23

Chest xray to rule out lung metastases [UpToDate: Given the propensity for lung metastases, chest imaging is recommended for newly diagnosed patients with soft tissue sarcoma of the extremity/trunk. While CT scan is often preferred due to its greater sensitivity in detecting small lung nodules, it is unknown whether this provides benefit over chest X-ray (CXR) alone. Both modalities are considered highly appropriate for this purpose by the American College of Radiology.]

Answer 24

XRT or intra-lesional vinblastine [UpToDate: All forms of KS, including CKS, are very sensitive to radiotherapy (RT). However, because of the multifocal nature of the disease, the persistence of HHV-8 even with successful local lesion control, and the tendency for new lesions to develop in nonirradiated areas, there is no consensus as to the place of RT in the therapeutic armamentarium (particularly when to choose RT over systemic therapy) or the optimal radiation technique. Intralesional injection of chemotherapy (most often vinblastine, but sometimes bleomycin) leads to local regression of cutaneous KS lesions. In our experience, such injections, while capable of eradicating injected tumors, can be painful and lead to local scarring.]

Answer 25

Acral lentiginous [UpToDate: Acral lentiginous melanomas are the least common variant of radial growth phase melanomas, comprising fewer than 5 percent of all melanomas. They arise most commonly on palmar, plantar, subungual, and occasionally, mucosal surfaces. The acral lentiginous subtype is the most common type of malignant melanoma among Asians and dark-skinned individuals, with a particular predilection for the soles of the feet. Clinically, an acral lentiginous melanoma appears as a dark brown to black, unevenly pigmented patch. Areas of regression manifest as foci of gray-white discoloration. If a lesion becomes raised or develops ulceration, the likelihood of invasion should be considered. However, some tumors that invade along the peri-eccrine adventitial dermis may appear flat clinically even if they have encroached upon subcutaneous adipose tissue.]

Answer 26

Imiquimod, cautery ablation, topical 5-FU, and regular biopsies to rule out cancer [Wide local excision should be avoided if possible because it is associated with HPV and has a high recurrence rate.] [UpToDate: Topical chemotherapy with topical 5-fluorouracil (5-FU) or imiquimod and photodynamic therapy are additional treatment options for patients with Bowen's disease (cutaneous SCC in situ).]

Answer 27

Intra-abdominal desmoid tumor [UpToDate: Most desmoids arise sporadically, although between 5 and 15 percent are associated with familial adenomatous polyposis (FAP). FAP is caused by mutations in the APC (adenomatous polyposis coli) gene, located on chromosome 5q21-q22.]

Answer 28

10% lifetime risk [UpToDate: Congenital melanocytic nevi (CMN) are classically defined as melanocytic nevi present at birth or within the first few months of life. These occur in 1 to 2 percent of newborn infants, and large or giant CMN occur in approximately 1 of 20,000 births. For patients with large CMN, the risk of developing melanoma (cutaneous or extracutaneous) is estimated to be approximately 2 to 5 percent over a lifetime, with most melanomas occurring in the first five years of life. Atypical nevi are benign, acquired melanocytic neoplasms that share some of the clinical features of melanoma, such as asymmetry, irregular borders, multiple colors, and diameter \>5 mm. The terms "atypical nevi" and "dysplastic nevi" are clinically used interchangeably, although in theory a dysplastic nevus refers to a histologic diagnosis. Although atypical nevi are benign lesions, they are strong phenotypic markers of an increased risk of melanoma, especially in individuals with numerous nevi and/or a family history of melanoma. In a meta-analysis of observational studies, the relative risk of melanoma associated with atypical nevi was 1.5 (95% CI 1.3-1.6) for the presence of a single atypical nevus and 6.36 (95% CI 3.80-10.33) for five atypical nevi versus none.]

Answer 29

The epidermis [Basal epithelial cells and hair follicles.] [UpToDate: Basal cell carcinoma (BCC) is a common skin cancer arising from the basal layer of epidermis and its appendages. These tumors have been referred to as "epitheliomas" because of their low metastatic potential. However, the term carcinoma is appropriate, since they are locally invasive, aggressive, and destructive of skin and the surrounding structures including bone.]

Answer 30

1. Large 2. Grow rapidly 3. Painless [Most common presentation is an asymptomatic mass. Can also present with GI bleeding, bowel obstruction, or neurologic deficit.]

Answer 31

Palliation [Best treatment is HAART (treatment for AIDS).] [UpToDate: Given the absence of treatments capable of eradicating latent HHV-8 infection, one can question whether any form of Kaposi sarcoma, including Classic Kaposi sarcoma (CKS), can be considered "curable". Nonetheless, a number of strategies have been used to manage CKS, with the major therapeutic goals of achieving symptom palliation, alleviating lymphedema, improving function, decreasing the size of cutaneous or visceral lesions, and delaying or preventing disease progression. The following represents our general approach: We suggest observation rather than specific treatment for patients who have a limited number of asymptomatic lesions that do not impair function (Grade 2C). Symptoms related to limited lower extremity edema can be alleviated in many patients with elastic compression stockings. For patients who have limited volume disease causing symptoms (eg, bleeding or chafing against clothes) or cosmetic disfigurement, we suggest local treatment rather than observation or systemic chemotherapy (Grade 2C). The choice of modality (radiation therapy, excision, cryotherapy, laser ablation) depends on a number of factors, including the site and extent of the disease involvement as well as clinician and patient preference. When there is an indication for chemotherapy we suggest pegylated liposomal doxorubicin (PLD) as the first line regimen, in the absence of a cardiac contraindication (Grade 2C). For patients who have progressed while receiving PLD or for those who do not respond to PLD, there are several options for second-line therapy in those who retain an adequate performance status. The available options include a single agent taxane, oral etoposide, vinblastine, or gemcitabine. No one regimen can be recommended over any other. The decision must be individualized, taking into consideration patient age, accompanying comorbidity, and clinician and patient preference.]

Answer 32

Melanocytes have dendritic processes that transfer melanin to neighboring keratinocytes via melanosomes [The density of melanocytes is the same among races; the difference is in melanin production.]

Answer 33

Basal cell carcinoma

Answer 34

* Leiomyosarcoma * Liposarcoma [UpToDate: Retroperitoneal STS (RPS) are relatively uncommon, constituting only 10 to 15 percent of all STS. The average annual incidence of RPS is approximately 2.7 cases per million population. Patients usually present in their 50s, although the age range is broad. The frequency is approximately equal in men and women. Approximately 80 percent of the neoplasms that arise within the retroperitoneal space are malignant. Furthermore, the majority of patients who present with a primary retroperitoneal, extravisceral, unifocal soft tissue mass will be found to have a sarcoma. In adults, the most common histologic types of RPS are liposarcomas and leiomyosarcomas, followed by undifferentiated/unclassified STS (a subset of which are pleomorphic undifferentiated sarcomas). Historically, undifferentiated/unclassified STS were included within the older term "malignant fibrous histiocytoma." A variety of other histologic types may be observed, but they are much less common in the retroperitoneum than in other primary sites. Approximately one-half of all RPS are high-grade tumors, although this varies according to histology. The majority of retroperitoneal liposarcomas are low- to intermediate-grade lesions.]

Answer 35

1. Poor differentiation 2. Greater depth 3. Recurrent lesions 4. Immunosuppression

Answer 36

* If small, excise * If large, biopsy and observe [Always biopsy if not going to excise in order to rule out cancer. It will involute spontaneously over a few months.] [UpToDate: We suggest surgical excision as the first-line treatment for KA (Grade 2C). Surgical excision provides pathologic confirmation of tumor removal. Conventional surgical excision may be performed on most lesions. Lesions in areas where tissue sparing is desired, such as the central face, can be treated with Mohs surgery. Other effective options for the treatment of KA include electrodesiccation and curettage (ED&C), intralesional pharmacologic therapy, radiation therapy, and topical therapy. A disadvantage of these interventions compared with surgical excision is the lack of histopathologic confirmation of tumor removal. For patients who elect to defer therapy and prefer to wait for lesion spontaneous resolution, close clinical follow-up should be implemented. Spontaneous resolution may take several months or longer. If lesions deviate from the expected clinical course, surgical excision to confirm the diagnosis is indicated. The local therapies commonly utilized for the management of solitary KA may be impractical for patients with hundreds or thousands of lesions. For patients with numerous KAs, we suggest treatment with oral retinoid therapy (Grade 2C).]

Answer 37

Ruffini's endings

Answer 38

Basal cell carcinoma

Answer 39

5% of skin cancers and 65% of deaths [SCORE: Even though melanoma accounts for less than 2% of skin cancer cases, it is currently the fifth most common cancer in men and the seventh most common cancer among women in the United States. Melanoma also causes the majority of skin cancer–related deaths.2 For 2014, the American Cancer Society estimated 76,100 new cases of melanoma in the United States, with approximately 9170 deaths. These numbers may continue to climb; melanoma incidence has steadily increased worldwide during the last 50 years.]

Answer 40

* In situ: 0.5-1 cm margin * _\<_ 1 mm in depth: 1 cm margin * 1.1-2 mm in depth: 1-2 cm margin * \> 2 mm in depth: 2 cm margin [UpToDate: Our approach is to resect melanomas \<1 mm thick (T1) with a 1 cm margin of normal tissue. For melanomas 1 to 2 mm thick (T2 lesions), we use a 2 cm margin of normal tissue if this is feasible without the need for a skin graft; a 1 to 2 cm margin may be adequate if anatomically constrained based upon the location of the lesion.]

Answer 41

Erythema and pain without skin loss

Answer 42

Squamous cell carcinoma in situ that is associated with HPV [10% turn into invasive squamous cell carcinoma.] [UpToDate: Cutaneous SCC in situ typically presents as a well-demarcated, scaly patch or plaque. Lesions are often erythematous but can also be skin colored or pigmented. SCC in situ lesions tend to grow slowly, enlarging over the course of years. Unlike the inflammatory disorders that may resemble SCC in situ, lesions are usually asymptomatic.]

Answer 43

* A: Asymmetry (angulations, indentations, notching, ulceration, bleeding) * B: Borders that are irregular * C: Color change (ie darkening. Blue color is the most ominous) * D: Diameter increase * E: Evolution over time

Answer 44

50% (15% according to UpToDate) [Arise from embryonic mesoderm.] [UpToDate: Among children, who account for 10 to 15 percent of all cases of soft tissue sarcoma, the "small round blue cell" sarcomas predominate (eg, Ewing sarcoma, embryonal RMS, and peripheral primitive neuroectodermal tumor [PNET]).]

Answer 45

Back, neck, between shoulders [Lipomas are common but rarely malignant.] [UpToDate: Lipomas present as soft, painless subcutaneous nodules ranging in size from 1 to \>10 cm. They occur most frequently on the trunk and upper extremities and can be round, oval, or multilobulated. Frequently, patients may have more than one lipoma, and occasionally they may have a genetic condition (familial multiple lipomatosis) characterized by the development of multiple lipomas in several family members. Malignant transformation of a lipoma into a liposarcoma is rare.]

Answer 46

Metaphyseal cells [Usually occurs in children.] [UpToDate: Bone sarcomas account for approximately 6 percent of all childhood cancers, and osteosarcomas account for approximately 3 percent of childhood cancers overall. However, osteosarcoma is the most common primary malignant bone tumor affecting children and young adults. Osteosarcomas comprise 56 percent of all bone cancers in individuals under the age of 20, while Ewing sarcoma accounts for 34 to 36 percent, and chondrosarcomas are responsible for less than 10 percent. In children, the peak incidence is between 13 and 16 years of age, a time that appears to coincide with the adolescent growth spurt. For unclear reasons, osteosarcomas are more common in boys than in girls, and in blacks and other races compared with Caucasians. The most common sites of osteosarcoma in children are the metaphyses of long bones, especially the distal femur, proximal tibia, and proximal humerus.]

Answer 47

The lungs [Melanoma is the most common metastasis to the small bowel.]

Answer 48

Partial skin loss with yellow debris [Treatment: Local measures, keep pressure off.]

Answer 49

Childhood rhabdomyosarcoma [UpToDate: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood, and responsible for approximately one-half of all soft tissue sarcomas in this age group. However, they are rare, representing only 3 to 4 percent of pediatric cancers overall. Approximately 350 new cases are diagnosed in the United States each year, and the annual incidence in children, adolescents, and young adults under the age of 20 is 4.3 cases per one million. Two-thirds of cases are diagnosed in children younger than six years of age, and there is a small male predominance (male to female ratio between 1.3 and 1.5). The incidence in African American patients is higher than in whites, most notably in 15 to 19 year olds. The incidence appears to be lower in Asians when compared with predominantly Caucasian populations.]

Answer 50

Terminal aspect of the digit [Benign] [UpToDate: Glomus tumor is a rare, benign neoplasm composed of cells resembling smooth cells of the normal glomus body. It is usually located in areas of the skin that are rich in glomus bodies (eg, the subungual regions of digits or the deep dermis of the palm, wrist, forearm, and foot). Glomus tumors of the fingers and toes occur in approximately 5 percent of patients with neurofibromatosis type 1 (NF1) and are considered NF1-associated neoplasms. Glomus tumor presents as a pink or purple vascular papule or nodule associated with paroxysmal pain, cold sensitivity, and tenderness. Subungual glomus tumors are particularly painful. Nail dystrophy and/or a bluish-red flush are typically seen.]

Answer 51

Liquid nitrogen [Warts are viral in origin, contagious, autoinoculable, and can be painful.] [UpToDate: For patients with common or plantar warts, we suggest topical salicylic acid as first-line treatment (Grade 2A). Cryotherapy is an alternative first-line treatment; however, tolerance for associated pain and other side effects (eg, hypopigmentation) limits use in some patients. The best approach to patients with severe or refractory cutaneous warts is unclear. Topical immunotherapy with contact allergens, intralesional bleomycin, or topical or intralesional 5-fluorouracil may be useful for such patients. Flat warts are usually treated with cryotherapy or topical agents such as tretinoin, imiquimod, or 5-fluorouricil. Filiform warts are often most easily treated with cryotherapy or surgery.]

Answer 52

Glomus cell tumor [Benign] [UpToDate: Glomus tumor is a rare, benign neoplasm composed of cells resembling smooth cells of the normal glomus body. It is usually located in areas of the skin that are rich in glomus bodies (eg, the subungual regions of digits or the deep dermis of the palm, wrist, forearm, and foot). Glomus tumors of the fingers and toes occur in approximately 5 percent of patients with neurofibromatosis type 1 (NF1) and are considered NF1-associated neoplasms. Glomus tumor presents as a pink or purple vascular papule or nodule associated with paroxysmal pain, cold sensitivity, and tenderness. Subungual glomus tumors are particularly painful. Nail dystrophy and/or a bluish-red flush are typically seen.]

Answer 53

Yes [XRT and chemotherapy may be of limited benefit for inoperable disease, metastases, or neuro/lymphatic/vessel invasion.]

Answer 54

Tuberous sclerosis [UpToDate: TSC is characterized by the development of a variety of benign tumors in multiple organs, including the brain, heart, skin, eyes, kidney, lung, and liver. In addition, there is an increased risk of malignancy in TSC. Nearly all patients with TSC have one or more of the skin lesions characteristic of the disorder. Most patients with TSC have epilepsy, and one-half or more have cognitive deficits and learning disabilities; other common manifestations include autism, behavioral problems, and psychosocial difficulties. Collectively, these are termed TSC-associated neuropsychiatric disorders (TAND). These problems are usually associated with brain lesions including glioneuronal hamartomas (also called tubers), periventricular giant cell astrocytomas, and abnormalities of cerebral white matter detected on neuroimaging studies. However, there is a wide variety of phenotypes between and within families regarding the number and severity of TSC manifestations.]

Answer 55

By 1. Local recruitment 2. Thinning of the dermis and epidermis 3. Mitosis

Answer 56

Yes if no other evidence of systemic disease [This gives the best chance for survival. Alternatively can palliate with XRT.] [UpToDate: For appropriately selected patients with isolated, limited pulmonary metastases from soft tissue sarcoma, we suggest pulmonary metastasectomy rather than palliative systemic chemotherapy alone (Grade 2B). There is no consensus as to the optimal selection of surgical candidates; however, the following criteria are generally agreed upon: * No extrathoracic disease, pleural effusion or mediastinal/hilar adenopathy * The primary tumor is controlled or controllable * The patient is a medically appropriate candidate for thoracotomy and pulmonary resection * Complete resection appears feasible Although far fewer data are available, isolated hepatic metastases may be amenable to resection. There is no consensus as to appropriate eligibility requirements, and the decision to operate must be made on a case by case basis. In addition, as with pulmonary lesions, it is critical to consider the underlying histology of the tumor, the expected natural history, and expected responsiveness to systemic therapy when considering surgery as a component of the treatment plan for metastatic disease.]

Answer 57

Apocrine sweat glands [Usually occurs in the axilla and groin.] [UpToDate: The pathogenesis of hidradenitis suppurativa (acne inversa, HS/AI) is not fully understood. However, an explosion of research has led to greater understanding of the processes involved. As opposed to early theories that implicated apocrine glands as the primary contributors to HS/AI, most authors now support follicle-centered theories for the pathogenesis of HS/AI. Follicular occlusion, follicular rupture, and an associated immune response appear to be important events in the development of the clinical manifestations of HS/AI. The basic principles that underlie follicle-centered theories are reviewed below. Follicular occlusion is the most likely event responsible for the initial development of HS/AI lesions. Follicular occlusion appears to result from a hormonally-induced increase in ductal keratinocyte proliferation (follicular epithelial hyperplasia), leading to a failure of keratinocytes to shed properly from the follicular epithelium, causing follicular hyperkeratosis and plugging. Further study is necessary to explore the pathways that promote follicular occlusion in this disease. Proposed contributors have included the effects of hormones and nicotine on the follicular epithelium. In addition, we propose that anoxia within the follicular duct secondary to follicular epithelial hyperplasia contributes to the disruption of normal terminal differentiation of follicular keratinocytes, leading to follicular plugging.]

Answer 58

Krause's end-bulbs

Answer 59

Venous thrombosis

Answer 60

10% [Other risk factors for melanoma include fair skin, easy sunburn, intermittent sunburns, previous skin cancer, previous XRT.]

Answer 61

50% [UpToDate: Soft tissue sarcomas occur at all anatomic body sites, but the majority are in the extremities. The anatomic distribution of soft tissue sarcomas in 4550 adults reviewed by the American College of Surgery was as follows: * Thigh, buttock, and groin – 46 percent * Upper extremity – 13 percent * Torso – 18 percent * Retroperitoneum – 13 percent * Head and neck – 9 percent Some histologic types of soft tissue sarcoma have a predilection for certain anatomic sites. As an example, while only 14 percent of all soft tissue sarcomas present in the upper extremity, 40 to 50 percent of all epithelioid sarcomas arise on the forearm and finger.]

Answer 62

Keratoacanthoma [Debris is keratin. It is not malignant but can be confused with squamous cell carcinoma. It involutes spontaneously over months.] [UpToDate: Keratoacanthoma (KA) is a cutaneous tumor that most commonly presents as a dome-shaped nodule with a central keratin-filled crater. KA most frequently develops on hair-bearing, sun-exposed skin. Middle-aged and elderly adults with fair complexions are most frequently affected. A distinguishing feature of KA is a clinical course characterized by rapid initial growth followed by a variable period of lesion stability and subsequent spontaneous resolution. The recognition of this unique characteristic in a tumor with clinical and histopathologic features that closely resemble cutaneous squamous cell carcinoma has led to significant debate over the classification of this lesion.]

Answer 63

Melanin [UV radiation damages DNA and repair mechanisms.]

Answer 64

In the basal layer of epidermis

Answer 65

This region will undergo XRT later [Post-op XRT for high-grade tumors, close margins, or tumors \> 5 cm.] [UpToDate: The combination of surgery and radiation therapy (RT) achieves better local control than either modality alone for the majority of STS for which adjuvant therapy is indicated (ie, high grade tumors \>5 cm where wide or radical margins are not possible short of amputation). In addition, the use of adjunctive RT maximizes functional outcomes while avoiding the significant morbidity and cosmetic deformity associated with radical resection. The use of intensity-modulated RT (IMRT) may improve local control and reduce morbidity compared with three-dimensional conformal radiation therapy (3D-CRT), and is preferred for patients in whom target volume coverage is not homogeneous and/or if normal tissues are not well spared with 3D-CRT.]

Answer 66

Melanoma [The most common location for distant melanoma metastases is the lung.]

Answer 67

Midline incision [With resection, try to preserve motor nerves and retain or reconstruct vessels.]

Answer 68

Anterior abdominal wall [Desmoid tumors have a high risk of local recurrence, but no distal spread.] [UpToDate: Most desmoid tumors present as a deeply seated painless or minimally painful mass with a history of slow growth. Intraabdominal desmoids can present with intestinal obstruction, bowel ischemia, or functional deterioration in an ileoanal anastomosis (typically in a patient who has undergone colectomy for familial adenomatous polyposis [FAP]). Desmoid tumors can develop at virtually any body site, but three main anatomic sites are described: trunk/extremity, abdominal wall, and intraabdominal (bowel and mesentery). In patients with FAP, intraabdominal desmoids predominate. In non-FAP-associated cases, the most commonly involved areas are the shoulder girdle, hip-buttock region, and the extremities, where the location is usually deep in the muscles or along fascial planes. Desmoid tumors may be multifocal at one site (typically the extremity), but they rarely occur at different regions in the same patient.]

Answer 69

Meissner's corpuscles

Answer 70

Staph and strep [UpToDate: The role of bacteria in HS/AI is controversial. Cultures from early, unruptured HS/AI lesions are usually sterile. Older and ruptured lesions and sinuses may demonstrate a wide variety of bacteria (eg, staphylococci, streptococci, Gram-negative rods, and anaerobic bacteria). Coagulase-negative staphylococci are frequently present. Positive cultures may represent contaminants from normal skin flora or secondary infection. One theory suggests that bacteria may contribute to HS/AI by promoting an inflammatory response, and a role for a bacterial biofilm in the persistence of the inflammatory process of HS/AI has been proposed. Additional studies may clarify the role of bacteria on the clinical course of HS/AI.]

Answer 71

Nodular melanoma [Most likely to have metastasized at the time of diagnosis.] [UpToDate: By definition, nodular melanomas are vertical growth phase melanomas. They constitute 15 to 30 percent of all melanomas. Clinically, a nodular melanoma appears as a darkly pigmented, pedunculated or polypoid nodule, although amelanotic variants are infrequently seen.]

Answer 72

Rhabdomyosarcoma [Head and neck sarcoma are hard to treat given difficulty in obtaining resection margins due to proximity of vital structures. Post op XRT for positive or close margins as negative margins may be impossible to obtain.] [UpToDate: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood, and responsible for approximately one-half of all soft tissue sarcomas in this age group. However, they are rare, representing only 3 to 4 percent of pediatric cancers overall. Approximately 350 new cases are diagnosed in the United States each year, and the annual incidence in children, adolescents, and young adults under the age of 20 is 4.3 cases per one million. Two-thirds of cases are diagnosed in children younger than six years of age, and there is a small male predominance (male to female ratio between 1.3 and 1.5). The incidence in African American patients is higher than in whites, most notably in 15 to 19 year olds. The incidence appears to be lower in Asians when compared with predominantly Caucasian populations.]

Answer 73

Kaposi's sarcoma [Kaposi's sarcoma is associated with immunocompromised state. Though it is the most common malignancy in AIDS, it is rarel the cause of death.] [UpToDate: AIDS-related or epidemic KS is the most common tumor arising in HIV-infected persons. KS is considered an AIDS-defining illness in the Centers for Disease Control and Prevention (CDC) guidelines. In the United States, KS was over 20,000 times more common in persons with AIDS than in the general population prior to the widespread use of potent antiretroviral therapy (ART), although its incidence has declined substantially since that time.]

Answer 74

Almost 100% risk of melanoma [UpToDate: The familial atypical multiple mole and melanoma (FAMMM) syndrome is an autosomal dominant condition characterized by a high number of common and atypical nevi (\>50) and history of melanoma in one or more first- or second-degree relatives. This syndrome is associated with mutations in the CDKN2A gene with reduced penetrance and variable expressivity. The CDKN2A gene is located on chromosome 9p21.3 and encodes the proteins which are involved in the regulation of critical cell cycle pathways. Patients harboring the specific CDKN2A mutation have an increased risk of early or multiple melanomas and other primary cancers, particularly pancreatic cancer.]

Answer 75

Squamous cell carcinoma [May have surrounding induration and satellite nodules.]

Answer 76

Li-Fraumeni syndrome [UpToDate: Li-Fraumeni syndrome is an inherited autosomal dominant disorder that is manifested by a wide range of malignancies that appear at an unusually early age. Li-Fraumeni syndrome is also known as the Sarcoma, Breast, Leukemia, and Adrenal Gland (SBLA) cancer syndrome. This cancer predisposition syndrome is inherited as an autosomal dominant disorder and is associated with abnormalities in the tumor protein p53 gene (TP53). The Li-Fraumeni syndrome was originally described in 1969, based upon the identification of second soft tissue sarcomas in five families with index cases of rhabdomyosarcoma. These observations were subsequently extended to 24 kindreds in which 151 individuals developed cancer. These patients were characterized by a variety of bone and soft tissue sarcomas and breast cancer, approximately 80 percent of which occurred prior to 45 years of age. Other tumors that occurred disproportionately in this series included brain tumors, leukemia, and adrenocortical carcinoma, and there were six patients with cancer that was linked to antecedent radiation therapy. Other reports have confirmed the high frequency of early age at onset in patients with Li-Fraumeni syndrome, as well as the wide range of cancers that are seen.]

Answer 77

The ability to completely remove the tumor [UpToDate: Surgical resection has traditionally been the only potentially curative treatment for a localized RPS. The ability to perform a complete surgical resection at the time of initial presentation is the most important prognostic factor for survival. The usual reasons for unresectability are extensive vascular involvement or the presence of multiple peritoneal implants.]

Answer 78

Mass \< 4 cm: Excisional biopsy Mass \> 4 cm: Longitudinal incisional biopsy [Need to eventually resect biopsy skin site if biopsy shows sarcoma.] [UpToDate: Our preferred method of obtaining tissue is with a core needle biopsy if technically feasible. Core needle biopsy is considered the preferred method to achieve an initial biopsy in most cases due to its low incidence of complications and high diagnostic accuracy. In a study of 530 patients with suspected soft tissue tumors, core needle biopsy differentiated malignant soft tissue sarcomas from benign soft tissue tumors in 97.6 percent of patients. Histologic grade was accurately determined in 86.3 percent of patients, and the subtype was accurately identified in 88 percent. If incisional biopsy is required, it should be carefully planned and performed by the surgeon who will be doing the definitive resection. Open biopsy incisions should be placed longitudinally along the extremity so that the scar can be resected along with the tumor at the time of definitive surgical resection. Adequate hemostasis is important to prevent dissemination of tumor cells.]

Answer 79

Squamous cell carcinoma [Risk factors for squamous cell skin carcinoma include: Actinic keratoses, xeroderma pigmentosum, Bowen's disease, atrophic epidermis, arsenics, hydrocarbons (coal tar), chlorophenols, HPV, immunosuppression, sun exposure, fair skin, previous XRT, previous skin cancer.]

Answer 80

Staging is based on grade rather than size [UpToDate: The most widely used staging system for soft tissue sarcomas is the TNM system developed by the International Union Against Cancer (UICC) and the American Joint Committee on Cancer (AJCC). The system uses tumor size (T), depth (superficial or deep), lymph node involvement (N), presence or absence of distant metastases (M), and histologic grade (G) in determining the stage grouping for soft tissue sarcomas.]

Answer 81

Surgery and doxorubicin-based chemotherapy [Similar to treatment for other soft tissue sarcomas.] [UpToDate: The treatment of rhabdomyosarcoma (RMS) has evolved considerably over the past several decades. Using modern combined modality therapy, over 70 percent of children with localized RMS can now be cured. Modern treatment includes chemotherapy for primary cytoreduction and eradication of both macroscopic and microscopic metastatic disease, surgery if feasible, and radiation therapy (RT) to control microscopic local residual disease. The specific treatment regimen depends on the estimated risk of a disease recurrence, which is based upon a variety of clinicopathologic prognostic factors, an approach termed risk-adapted therapy. We recommend complete excision for localized disease as long as functional and/or cosmetic results are acceptable (Grade 1A). If complete resection is not feasible, or if disease involves the orbit, vagina, bladder, or biliary tract, patients are better approached with an initial diagnostic biopsy followed by induction (neoadjuvant) chemotherapy then definitive local therapy. We recommend RT to enhance local control in patients with residual microscopic or gross disease following surgery (Grade 1B). The value of this modality for Clinical Group (CG) I alveolar tumors remains to be determined, but in the United States this is considered standard of care. We recommend chemotherapy in addition to local therapy for all patients with RMS (Grade 1A). The choice of regimen depends on the estimated risk of a disease recurrence.]

Answer 82

40% [Soft tissue sarcoma has a poor overall prognosis because of late diagnosis, difficulty with total resection, and difficulty getting XRT to pelvic tumors.] [UpToDate: In a series derived from Memorial Sloan-Kettering Cancer Center, five-year rates disease-free survival for stage I, II, and III disease were 86, 72, and 52 percent. The corresponding values for overall survival were 90, 81, and 56 percent. In a retrospective review of 1225 patients with localized soft tissue sarcoma treated with surgery and radiation therapy at M D Anderson Cancer Center, five-year rates of metastasis-free survival were 98, 85, and 64 percent for grade 1, 2, and 3 tumors respectively.]

Answer 83

Yes [Resection of metastases has provided some patients with long disease-free intervals and is the best chance for a cure.] [UpToDate: Patients with oligometastatic disease (ie, limited to one or a limited number of sites) should be evaluated for the possible surgical metastasectomy. Although widespread metastatic disease develops in most cases, complete resection of metastatic disease is associated with prolonged survival in some cases. Systemic therapy is indicated for patients in whom surgical metastasectomy is not appropriate. The primary systemic therapy approaches for patients with metastatic melanoma are checkpoint-inhibitor immunotherapy (primarily the anti-PD1 antibodies pembrolizumab and nivolumab in combination with the anti-CTLA4 antibody ipilimumab) and targeted therapy against the mitogen activated protein kinase (MAPK) pathway. The appropriate choice and sequence of treatments for an individual patient is based upon the extent of disease, molecular characteristics of the tumor (presence of a driver mutation in BRAF or KIT), and patient performance status and comorbidity.]

Answer 84

Yes for tumors \> 10 cm as it may allow for a limb-sparing resection [UpToDate: Theoretical advantages to neoadjuvant approaches to therapy include tumor cytoreduction, immediate treatment of micrometastases, and an early indication as to the effectiveness of chemotherapy/radiotherapy. Cytoreduction may allow less radical surgical resection to be performed, and this approach is often considered in patients with large extremity sarcomas, particularly if the patient is a borderline candidate for limb salvage surgery. Most often, when induction therapy is considered for a patient with a large or recurrent extremity sarcoma, particularly if limb salvage is an issue, radiotherapy is chosen with or without chemotherapy. Adjuvant radiotherapy with and without chemotherapy is discussed in detail elsewhere.]

Answer 85

Desmoid tumor [Desmoids can occur during or following pregnancy and can also occur after trauma or surgery.] [UpToDate: Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. The term "desmoid" originates from the Greek word "desmos," meaning band or tendon-like, and was first applied in the 1800s to describe tumors with a tendon-like consistency. Although they lack the capacity to establish metastases, desmoids are locally aggressive and have a high rate of recurrence even after complete resection. Tumor-related destruction of vital structures and/or organs can be fatal, particularly when these tumors arise in patients with familial adenomatous polyposis (FAP; Gardner's syndrome).]

Answer 86

Near the knee [UpToDate: Bone resections fall into one of three categories, depending upon the anatomic site and the extent of the involved bone that needs to be excised. Because most bone sarcomas arise in the metaphysis of the long bones near a joint, the majority of resections, for tumors in the lower extremity, include both the segment of tumor-bearing bone and the adjacent joint (osteoarticular resection). Most often, the incision is performed through the joint (intraarticular resection); however, when the tumor extends along the joint capsule or ligamentous structures or invades the joint, the entire joint can be resected (extraarticular resection) to avoid cutting through tumor.]

Answer 87

Superficial spreading melanoma [UpToDate: Superficial spreading melanoma is the most common subtype, comprising approximately 75 percent of all malignant melanomas. One-fourth of superficial spreading melanomas are found in association with a preexisting nevus, such as a dysplastic or congenital nevus, while the majority appear to arise de novo. These melanomas derive their name from histologic evidence of lateral (radial) growth for a period of time before vertical (invasive) growth occurs. Although superficial spreading melanoma has a predilection for the back in men and lower extremities in women, it can occur in any anatomic location, and at any age. Clinically, the superficial spreading type of melanoma appears as a variably pigmented plaque with an irregular border, ranging from a few millimeters to several centimeters in diameter. In fully evolved lesions, multiple shades of red, tan, brown, blue, black, gray, and white can be appreciated. The red zones are thought to represent areas of vessel ectasia and inflammation, and the white and gray regions reflect amelanotic or regressed foci. An asymmetric flare at the lesion periphery represents the advancing border of proliferating, neoplastic melanocytes.]

Answer 88

Lungs [Sarcomas spread hematogenously rather than lymphatically. Metastasis to nodes is rare.] [UpToDate: For most STS of the extremity, chest wall, or head or neck, the primary metastatic site is the lung. However, there are exceptions. Extrapulmonary metastases to the retroperitoneum, spine, and paraspinous soft tissues predominate with myxoid/round cell liposarcomas, although lung metastases develop eventually in almost all.]

Answer 89

Gardner's syndrome [UpToDate: Shortly after discovery of the adenomatous polyposis coli (APC) gene, the gene responsible for familial adenomatous polyposis (FAP), it became apparent that both FAP and Gardner syndrome (GS) arose from APC mutations. FAP is characterized by hundreds to thousands of colonic adenomatous polyps that most often emerge in the second and third decades of life. Colon cancer is inevitable if the colon is not removed. Polyposis is also usually observed in the stomach, duodenum, and small bowel, although the cancer risk in these locations is far less than in the colon. Inheritance is autosomal dominant with near complete penetrance of the gastrointestinal phenotype but with variable penetrance of the extraintestinal manifestations of the disease. GS cannot be separated from FAP when considering studies that describe its overall prevalence. Estimates for the prevalence of the combined syndromes vary from 1 in 6850 to 1 in 31,250 people (2.29 to 3.2 cases per 100,000 persons). Prevalence appears fairly constant throughout the world with men and women affected equally. Twenty to 30 percent of newly diagnosed cases, ie, those not belonging to previously identified families, appear to represent new mutations. New cases may also arise from mosaic inheritance, which implies that a mutation occurred in parent's sperm or egg cells, but not in other cells of the body, so the parent did not have clinical disease. It was once believed that GS patients exhibited fewer and more distinct colonic polyps. However, continued study has demonstrated that the gastrointestinal polyp and cancer phenotypes, although variable, are identical for both GS and FAP. Colonic polyp number depends to some degree on where the mutation occurs in the APC gene. Mutations in the center of the gene (often called the mutation cluster region) give rise to dense polyposis, with 5000 or more colonic polyps when the disease is fully developed. If mutations occur proximal or distal to this central gene location, colonic polyps average approximately 1000 with full expression. Mutations in the extreme proximal or distal locations of the APC gene, or in certain areas of exon none, are associated with many fewer polyps (often less than 100). This clinical variation is referred to as attenuated FAP. Extraintestinal growths do not correlate with polyp density but have some correlation with mutation location. The common extraintestinal manifestations associated with GS have been described in approximately 20 percent of patients with FAP. However, many more patients with FAP have these features if they undergo detailed physical and radiologic examinations. Thus, the difference between FAP and GS is somewhat semantic and GS is usually considered a subset of FAP. On the other hand, the term GS continues to be commonly applied, particularly in families that exhibit frequent and obvious extraintestinal lesions.]

Answer 90

Neuroendocrine tumor [Very aggressive, malignant tumor with early regional and systemic spread. It is red to purple in color and appears as a papulonodule or indurated plaque.] [UpToDate: Merkel cell carcinoma (MCC) of the skin is a rare, aggressive cutaneous malignancy. MCC typically presents in older patients with light skin tones as a rapidly growing, painless, firm, non-tender, shiny, flesh-colored or bluish-red, intracutaneous nodule. Ulceration and crusting are infrequent. MCCs range in size from less than 1 cm to over 2 cm and are most often located in sun-exposed areas.]

Answer 91

Neurofibromatosis [UpToDate: There are three major clinically and genetically distinct forms of neurofibromatosis: neurofibromatosis types 1 and 2 (NF1 and NF2) and schwannomatosis. NF1, also known as von Recklinghausen disease, is the most common type. The hallmarks of NF1 are the multiple café-au-lait macules and neurofibromas. The condition is called segmental NF1 when clinical features are limited to one area of the body.]

Answer 92

3 cm margins and at least 1 uninvolved fascial plane [Attempts should be made to perform a limb-sparing operation.] [UpToDate: The goal of the surgical resection should be to widely resect the tumor with negative margins, meaning a cuff of normal tissue completely surrounding the tumor. The amount (thickness) of the uninvolved tissue necessary is debatable (traditionally 1 cm margins were recommended) and the assessment of the margin is difficult at best when the specimen leaves the wound. One cm margins seldom occur in reality, especially around neurovascular structures, and it is generally accepted that the type of tissue is important: thinner (1 to 2 mm) margins of fascia are likely adequate, whereas wider margins that consist of fat or muscle are suggested.]

Answer 93

Merkel cell carcinoma (neuroendocrine tumor)

Answer 94

Liposarcoma [#1 is malignant fibrous histiosarcoma (also known as pleomorphic undifferentiated sarcoma).]

Answer 95

1. Most common and has completely mature epidermis with creamy keratin material: **Epidermal inclusion cyst** 2. In scalp, no epidermis: **Trichilemmal cyst** 3. Over tendons, usually over wrist; filled with collagen material: **Ganglion cyst** 4. Midline intra-abdominal and sacral lesions usually; need resection due to malignancy risk: **Dermoid cyst** 5. Congenital coccygeal sinus with ingrown hair; gets infected and needs to be excised: **Pilonidal cyst**

Answer 96

Keratinocytes originate from the basal layer [Keratinocytes provide a mechanical barrier. The epidermis is primarily cellular.]

Answer 97

0.5-1.0 cm margins [High risk cancers can be treated with Mohs surgery when trying to minimize the area of resection such as with lesions on the face.] [UpToDate: Surgical excision is appropriate for both low-risk and high-risk cutaneous SCCs and is frequently used for treatment. Excision usually can be performed in an outpatient setting under local anesthesia. Surgical excision is well tolerated and effective, and the completeness of the procedure can be evaluated through histologic assessment of the specimen's margins. The efficacy of surgical excision for cutaneous SCC is supported by observational studies. A systematic review that identified 12 observational studies that assessed tumor recurrence following surgical excision of invasive cutaneous SCC (1144 patients) found local recurrence rates ranging from 0 to 15 percent, with a pooled average local recurrence rate of 5.4 percent (95% CI 2.5-9.1 percent). Recommendations for surgical margins vary depending upon the risk for local recurrence. Well-defined, small (\<2 cm) cutaneous SCCs lacking any high-risk features require a 4 mm margin of normal tissue around the visible tumor to result in a 95 percent histologic cure rate. Surgical excision for high-risk cutaneous SCC is reviewed separately. In accordance with guidelines proposed by the NCCN, conventional surgical excision is appropriate only for high-risk tumors on the trunk or extremities that have a size greater than 2 cm as the only high-risk feature. Such tumors should be excised with a 1 cm margin and primary closure.]

Answer 98

CT chest/abdomen/pelvis, LFTs, and LDH [Physical exam of all possible draining lymph nodes should also be performed.]

Answer 99

* Man: Back * Woman: Legs