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MSS 2 > Pathology of Soft Tissue > Flashcards

Pathology of Soft Tissue Flashcards

1
Q

*** What is DERMATOMYOSITIS?

A
  • inflammatory disorder of the skin and skeletal muscle

- unknown etiology.

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2
Q

With what are some cases of dermatomyositis associated?

A
  • gastric carcinoma
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3
Q

What are the clinical features of dermatomyositis?

A
  • bilateral PROXIMAL muscle weakness (can’t raise arm to comb hair), but distal can develop late in the disease.
  • rash of the UPPER EYELIDS (HELIOTROPE RASH) or MALAR rash.
  • red papules on the elbows, knuckles, and knees (GOTTRON PAPULES).
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4
Q

What lab findings will you see in dermatomyositis?

A
  • increased creatinine kinase (enzyme found in muscle, which will leak out into the blood when damaged).
  • positive ANA and anti-Jo-1 antibody (hallmark of this disease).
  • PERIMYSIAL inflammation (CD4+ T cells) with PERIFASICULAR atrophy
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5
Q

How do you remember perimysial inflammation for dermatomyositis vs. endomysial inflammation for polymyositis?

A
  • peri= outside and closer to the skin (dermatomyositis)
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6
Q

How do you treat dermatomyositis?

A
  • corticosteroids
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7
Q

** What is POLYMYOSITIS?

A
  • inflammatory disorder of skeletal muscle.
  • resembles dermatomyositis clinically, but SKIN is NOT involved.
  • will see ENDOMYSIAL inflammation (CD8+ T cells) with necrotic muscle fibers.
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8
Q

** What is X-linked Duchenne muscular dystrophy? (PICMONIC)

A
  • degenerative disorder due to SPONTANEOUS DELETIONS of the DYSTROPHIN gene, characterized by muscle wasting and REPLACEMENT of SKELETAL MUSCLE with ADIPOSE tissue.
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9
Q

What is the role of dystrophin?

A
  • anchoring the muscle cytoskeleton to the extracellular matrix.
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10
Q

What is the largest gene in the human genome?

A
  • dystrophin gene
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11
Q

How do babies (usually boys bc X-linked recessive) present with Duchenne muscular dystrophy?

A
  • PROXIMAL muscle weakness at 1 year of age, then progresses to distal muscles.
  • CALF PSEUDOHYPERTROPHY
  • elevated serum CREATINE KINASE.
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12
Q

What causes death in Duchenne muscular dystrophy?

A
  • cardiac or respiratory failure; myocardium is usually involved.
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13
Q

*** What is Becker Muscular Dystrophy?

A
  • MUTATED dystrophin gene, rather than deleted

- milder disease.

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14
Q

** What is MYASTHENIA GRAVIS? (PICMONIC)

A
  • autoantibodies against the POSTSYNAPTIC ACETYLCHOLINE RECEPTOR at the neuromuscular junction.
  • more commonly seen in women.
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15
Q

What are the clinical features of myasthenia gravis?

A
  • muscle weakness that WORSENS with USE (bc you are using up the acetylcholine stores available) and improves with rest; classically involves the EYES, leading to PTOSIS and DIPLOPIA (double vision).
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16
Q

Will myasthenia gravis symptoms improve or worsen with acetylcholinesterase inhibitors?

A
  • IMPROVE bc there will be more acetylcholine around for a longer period of time.
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17
Q

** With what other condition myasthenia gravis associated?

A
  • thymic hyperplasia or thymoma

* thymectomy improves symptoms.

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18
Q

** What is LAMBERT-EATON SYNDROME? (PICMONIC)

A
  • antibodies against PRESYNAPTIC CALCIUM CHANNELS at the NMJ.
  • arises as a PARANEOPLASTIC SYNDROME (usually due to small cell lung carcinoma).
  • leads to IMPAIRED acetylcholine release bc firing of presynaptic Ca2+ channels is required for acetylcholine release.
19
Q

What are the clinical features of lambert-eaton syndrome?

A
  • PROXIMAL muscle weakness that IMPROVES with USE bc more calcium can kick off the antibody.
20
Q

Are eyes affected or spared in lambert-eaton syndrome?

A
  • spared
21
Q

Will anticholinesterase (acetylcholinesterase inhibitors) improve symptoms of lambert eaton syndrome?

A
  • NO bc there is no acetylcholine around.
22
Q

Does lambert-easton syndrome resolve with resection of the tumor?

A
  • YES
23
Q

*** What is a LIPOMA?

A
  • BENIGN tumor of ADIPOSE tissue.

- most common benign soft tissue tumor in adults.

24
Q

*** What is LIPOSARCOMA?

A
  • MALIGNANT tumor of ADIPOSE tissue.

- most common malignant soft tissue tumor in adults.

25
Q

What is the characteristic cell of liposarcoma?

A
  • lipoblast
26
Q

*** What is a RHABDOMYOMA?

A
  • BENIGN tumor of SKELETAL MUSCLE.
27
Q

*** With what is cardiac rhabdomyoma associated?

A
  • tuberous sclerosis
28
Q

*** What is a RHABDOMYOSARCOMA?

A
  • MALIGNANT tumor of SKELETAL MUSCLE.

- most common malignant soft tissue tumor in CHILDREN (EMBRYONAL).

29
Q

What is the characteristic cell in rhabdomyosarcoma?

A
  • rhabdomyoblast

* DESMIN positive

30
Q

** What is the most common site for rhabdomyosarcoma?

A
  • head and neck

* vagina is classic site in young girls.

31
Q

What is neurogenic muscle atrophy?

A
  • muscle atrophy due to deprivation of innervation

- symptoms may be milk weakness to respiratory failure.

32
Q

What is nodular fasciitis?

A
  • reactive fibroblastic proliferation of tissue.
  • comprised of plump immature fibroblastic cells.
  • simple excision is curative.
33
Q

What are fibromatoses?

A
  • group of benign soft tissue tumors, with a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence.
  • may be superficial or deep.
34
Q

** What are Fibrosarcomas?

A
  • malignant fibroblast tumors that are unencapsulated, infiltrative FISH FLESH MASSES.
  • HERRINGBONE pattern.
  • plump spindle shaped cells.
  • metastatsize HEMATOGENOUSLY.
35
Q

What is a fibrous histiocytoma (dermatofibroma)?

A
  • benign well defined mobile nodule in the dermis or subcutaneous tissue.
  • Tan papule.
  • made of interlacing spindle cells in the dermis. Histiocytes may be present in the spindle component.
  • a CLEAR ZONE separates the mass from the epidermis.
36
Q

How do you treat a fibrous histiocytoma (dermatofibroma)?

A
  • local excision
37
Q

What is dermatofibrosarcoma protuberans?

A
  • intermediate between benign fibrohistiocytic tumors and malignant fibrohistiocytomas.
  • polypoid and multinodular
  • slow growing lesion in dermis and subcutaneous tissue.
  • fibroblastic spindle shaped cells in a STORIFORM or CARTWHEEL pattern.
38
Q

What is malignant fibrous histiocytoma?

A
  • most common soft tissue sarcoma in adults and post-irradiation sarcoma.
  • deep tissue of extremities and retroperitoneal area.
  • highly aggressive.
39
Q

How does the ALVEOLAR rhabodmyosarcoma differ from the embryonal rhabdomyosarcoma?

A
  • alveolar has a network of fibrous septae that divide cells into clusters, which look like pulmonary alveoli.
  • cross striations are not common (where they are in embryonal).
40
Q

*** What is a Schwannoma?

A
  • benign tumor of Schwann cells. Grow next to the nerve but do NOT invade it.
  • involves cranial or spinal nerves. Within the cranium, most frequently involves cranial nerve VIII at the cerebellopontine angle (presents as LOSS of HEARING and tinnitis).
  • positive for S-100
  • bilateral tumors are seen in neurofibromatosis type 2.
  • loss of NF2 gene product (MERLIN)
41
Q

What are ANTONI A vs. B areas in Schwannomas?

A
  • Antoni A= dense areas containing spindle cells arranged into intersecting fasicle. Also have VEROCAY BODIES (nuclei next to nuclear free zones).
  • Antoni B= loose, hypocellular areas with spindle cells spread apart by extracellular matrix.
42
Q

Can Schwannomas compress nerves?

A
  • YES
43
Q

** What is a neurofibroma?

A
  • benign nerve sheath tumors composed of spindle cells.
  • Schwann cells, mixed with perineural-like cells, fibroblasts, mast cells, and CD34+ spindle cells.
  • also positive for S-100.
  • AXONS run through the neoplasm (won’t see this in Scwhannoma).
44
Q

What is a synovial sarcoma?

A
  • malignant tumor around the joint space, but does NOT arise from synovial cells; it arises from MESENCHYMAL cells around the joint.
  • RARE
  • BIPHASIC PATTERN= epithelial and spindle cell components.

MSS 2 (15 decks)

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