109: Appendage Tumors Flashcards
What are the classifications of cutaneous appendage tumors?
Cutaneous appendage tumors are classified into four main types: Eccrine, Apocrine, Follicular, and Sebaceous.
What is the clinical presentation of cutaneous appendage tumors?
The clinical presentation typically includes solitary or multiple dermal papules or nodules, epidermal changes, skin-colored, pink, or bluish hue, possible visibility of hair follicle orifice or central punctum, specific site or age predilections, and diagnosis usually requires biopsy and subsequent histopathology.
What are the histopathologic diagnosis aims for appendage tumors?
The aims include arriving at a specific diagnosis, distinguishing benign from malignant tumors, determining whether the tumor is low or high grade, and identifying tumors associated with genetic syndromes.
What are the management strategies for benign and malignant appendage tumors?
Management strategies include monitoring clinically, surgical excision, and ablative therapies for benign tumors; and surgical excision using conventional or Mohs micrographic techniques for malignant tumors.
What are the characteristics of normal eccrine glands?
Normal eccrine glands are found throughout the body, especially on palms and soles, composed of a secretory coil and a duct connecting to the epidermis.
What are the common clinical features of benign hidradenoma?
Commonly occurs in the fifth decade of life, affects both genders, can occur at all anatomic sites, typically presents as a solitary dermal nodule, color ranges from flesh-colored to red or bluish, and size ranges between 0.5 and 10.5 cm.
What is the etiology and pathogenesis of benign hidradenoma?
50% carry t(11;19) translocation resulting in a fusion between the MECT1 gene and the MAML2 gene, promoting tumorigenesis by inducing cAMP/CREB target genes.
What are the histopathological features of poroma?
Relatively well-circumscribed endophytic proliferations of cuboidal cells with basophilic round nuclei and eosinophilic cytoplasm, with ductal differentiation variably present.
What are the differential diagnoses for hidradenoma?
Differential diagnoses include squamous cell carcinoma, invasive adenocarcinoma, and metastatic renal cell carcinoma.
What is the management strategy for benign hidradenoma?
Complete excision with clear margins is recommended, with local recurrence about 10% if not completely excised.
What are the clinical features of poroma?
Typically affects middle-aged to elderly patients, presents as a single flesh-colored to red papule or nodule, and can appear on acral surfaces.
What is the likely diagnosis for a solitary dermal nodule on the head and neck region with ductal differentiation?
The likely diagnosis is benign hidradenoma, also known as nodular hidradenoma or eccrine acrospiroma.
What genetic translocation is associated with 50% of benign hidradenomas?
The t(11;19) translocation resulting in a fusion between the MECT1 gene and the MAML2 gene.
What is the diagnosis for a single, flesh-colored to red papule on the acral surface with ductal differentiation?
The diagnosis is poroma, a benign eccrine neoplasm.
What are the four subtypes of poroma?
The four subtypes are poroma, hidroacanthoma simplex, dermal duct tumor, and poroid hidradenoma.
What are the clinical features of cylindroma?
Cylindromas present as smooth, flesh-colored to red nodules, primarily in the head and neck region, and occur in adults with a female predilection.
What is the etiology and pathogenesis of cylindromas?
Cylindromas associated with Brooke-Spiegler syndrome are caused by mutations in the CYLD tumor-suppressor gene.
What is the histopathological characteristic of cylindromas?
Cylindromas are characterized by islands of basaloid cells arranged in a ‘jigsaw puzzle’ pattern, surrounded by eosinophilic basement membrane material.
What is the management strategy for cylindromas?
The treatment of choice is surgical excision with clear margins.
What are the clinical features of spiradenoma?
Spiradenomas typically present as single, pink, gray, or blue dermal nodules on the trunk, primarily affecting young to middle-aged adults.
What is the histopathological feature of spiradenomas?
Spiradenomas are multinodular, unencapsulated dermal neoplasms composed of basaloid cells arranged in cords in a trabecular pattern.
What is the management approach for spiradenomas?
The management typically involves surgical excision with clear margins.
What are the clinical features of syringomas?
Syringomas present as multiple, 1- to 3-mm, flesh-colored or yellow papules, primarily on the face.
What is the histopathological characteristic of syringomas?
Syringomas are well-circumscribed and composed of epithelial cells arranged in cords, nests, and small ductal structures.