Dermal and Subcutaneous Tumors – Part II

1. Dermal and Subcutaneous Tumors – Part II Michael Hohnadel March 2004

2. Kaposi’s Sarcoma Five Presentations: 1. Classic • Middle aged, European men • Red, violaceous nodules on toes or soles. • Coalesce to form plaques. Brawny edema • Later more widespread involvement esp soft palate. Periods of remission/involution. 2. African Cutaneous • Nodular infiltrating vascular masses of extremities • Men 20-50 yrs. Endemic to Africa. • Locally aggressive. • Massive edema of legs and bone involvement.

3. Kaposi’s Sarcoma 3. African Lymphadenopathic • Lymph nodes with/without skin in children <10. • Fatal in < 2years. 4. AIDS • Violaceous macules progress to papules, nodules and plaques. • Head, neck, trunk and mucous membranes. 5. Lymphoma / immunosuppressive TX. Resembles classic but more variable presentation.

4. Kaposi’s Sarcoma Classic Variety.

5. Kaposi’s Sarcoma HIV Associated.

6. Kaposi’s Sarcoma

7. Kaposi’s Sarcoma • Internal Involvement • G.I. Tract is site of internal involvement esp the small intestine. • Skeletal changes are diagnostic • Rarefaction, cyst, cortical erosion • In AIDS: • 25% cutaneous alone, • 29% viscous alone. • Ultimately, 70% will have viscous involvement of G.I. (50%), lungs (37%) or Lymph nodes (50%)

8. Kaposi’s Sarcoma • Etiopathogenesis • Proliferation of abnormal vascular endothelial cells. • Muliticentric origin • HHV-8 is strongly assoc. and predictive of K.S. in HIV infected individuals. • Histology • Large endothelial cells of capillaries protrude into lumen like buds. • Lesions with proliferation of capillaries and fibrosarcoma like tissue in varying proportions. • Late lesions: spindle cell proliferation with sarcoma like properties.

9. Kaposi’s Sarcoma Early lesion with dilated thin walled vascular vessels with protruding endothelial cells.

10. Kaposi’s Sarcoma Later, ‘hemangioma- like’ lesion.

11. Kaposi’s Sarcoma • Treatment: • Radiation. (all types are responsive) • For individual lesions: cryotherapy, vincristine, excision, laser ablation. Alitretinoin applied 2-4 times daily. • Systemic chemotherapy if >10 lesions / month or symptomatic visceral involvement. • Resistance to single chemo agents over time so combo with other modalities. • Course: Usually progresses slowly and is rarely the cause of death. (except African cutaneous variety.

12. Epithelioid Hemangioendothelioma • Solitary, slow growing papule on the extremities. Intermediate between angiosarcoma and hemangioma. • M>F. Frequently before age of 25 years. • Rare. • Histology: Dilated vascular channels, spindle cells. • TX: Wide excision with evaluation of regional nodes. 20-30% 2 year mortality.

13. Spindle cell Hemangioendothelioma • Child or young adult with Multifocal occurance of firm, blue nodules on extremity • Rare. May recur when excised. Retiform Hemangioendothelioma • Low grade angiosarcoma. Slow growing exophytic mass sub-Q nodule or even a plaque. • Rare • Extremities of young adults • Wide excision. Nodes may be affected locally but no deaths reported due to Mets.

14. Angiosarcoma 4 Clinical settings: 1.) Head and neck tumor of elderly • F:M = ratio 2:1 • Ill defined bluish nodule resembles a bruise often with an erythematous ring. Satellite nodules, bleeding are common. • TX: Complete excision with radiation. • Multicentric nature and rapid metastasis usually results in death in two years. 2.) In area of chronic lyphedema. • Classically after mastectomy (Stewart-Treves) • Appears 10 years after surgery in 0.45 % of pts. • Mets to lungs = death in 19-31 months. (6% -5 year)

15. Angiosarcoma 3.) Previously irradiated sites • Interval of tumor development depends on nature of lesion for which radiation was given: • Benign – 23 years to develop. • Malignant – 12 years. • Prognosis: death in 6-24 months. 4.) Miscellaneous subset.

16. Angiosarcoma

17. Angiosarcoma Infiltration of the dermis by ill-defined vascular spaces and hyperchromatic, atypical endothelial cells. Factor VIII staining is positive.

18. Fibrous Tissue Abnormalities Keloids • Firm, irregularly shaped, fibrous excrescence usually at a site of previous trauma. Claw-like projections overgrow wound boundary. • May occur at any body site. Most common site - sternal. • Histopathology: Growth of myofibroblast and collagen in the dermis with a whirl like arrangement of hyalinized bundles of collagen. There is a paucity of elastic tissue and thinning of the overlying papillary dermis. • DDX: Hypertrophic scar- No claw like extensions. Stays in wound boundaries. Often improves in 6 mo. • TX: Intralesional kenalog 20-40 mg/cc. Lasers. Excision with intralesional injections. Silicone sheeting.

19. Keloids Hypertrophic Scar at 5 months and 1 year

20. Spontaneous Keloids

21. Keloids Hypertrophic Scar Keloid Keloid

22. Dupuytren’s Contracture • Fibromatosis of the palmar aponeurosis. Plantar Fibromatosis is seen on soles. • Men 30-50 years. Multiple firm nodules in the palm, 1 cm in diameter, proximal to the 4th finger. Contractures develop with time. • Associations: Alcoholic cirrhosis, DM, epilepsy, plantar fibromatosis, Peyronies dx. Familiar predispositions. • TX: Early intralesional Triamcinolone, Surgery.

23. Dupuytren’s Contracture

24. Knuckle Pads • Well defined, round, plaque-like fibrous thickenings that develop over the proximal interphalangeal joints on toes and fingers. • May become 10-15mm in diameter and persist permanently. Remain freely mobile. • Assoc with Dupuytren’s contractures and some autosomal dominate familial cases have been reported. • Histology: Fibromas • TX: intralesional steroids.

25. Knuckle Pads

26. Peyronie’s Disease • Fibrous infiltration of intercavernous septum of the penis results in nodules and plaques. A fibrous chordee is produced with curvature of the on erection. • Assoc with Dupuytren’s contractor • TX: Intralesional Triamcinolone may be curative.

27. Peyronie’s Disease As Dr. Ladd would say: ‘Something just ain’t right…..’

28. Desmoid Tumor • Deep seated well circumscribed mass arising from muscular aponeurosis. • Most common on abdominal wall esp. in women during or after pregnancy. • May be fatal if invade or compress vital structures. Most dangerous are those of neck and intra-abdominal • TX: MRI to evaluate extent of tumor. Excision.

29. Aponeourotic Fibroma • Juvenile aponeurotic fibroma. • Slow growing cyst-like masses occurring on the limbs. • X-ray reveals Stippled calcification. Congenital Generalized Fibromatosis • Multiple firm dermal and subdermal nodules presenting at near birth. • 2 types of involvement: • Dermal and Skeletal (metaphyseal) – resolution 2 yrs. • Involvement of viscera - 80% mortality. If live past 4 mo. then regression.

30. Infantile Digital Fibromatosis • Asymptomatic, firm, red, 1 cm. diameter nodules on DIP of fingers or toes during the first year of life. No metastasis. • Whorled fascicles of spindle cells eosinophilic inclusion bodies • Surgical excision has high recurrence. • TX: Observation, hope for spontaneous resolution.

31. Infantile Digital Fibromatosis Whorled fascicles of spindle cells Eosinophilic inclusion bodies

32. Fibrous Harmartoma of infancy • Single dermal or sub-q nodule of upper trunk present at birth. • Excision. • Fibomatosis Colli • Fibrous proliferation infiltration sternocleidomastoid m. at birth. • Spontaneous remission in a few months. • Diffuse Infantile Fibromatosis • Multicentric fibrous infiltration of m. of arms, neck and shoulder area.

33. Giant Cell Tumor of Tendon Sheath • Firm 1-3 cm. nodule attaches to tendons of fingers hand and wrist (esp flexor). • Histopathology: • Lobules of densely hyalinized collagen. Giant cells with eosinophilic cytoplasm and variable nuclei. • TX • Excision. Recurs in 25%.

34. Giant Cell Tumor of Tendon Sheath Sheets of epithelioid histiocytes with a variable number of the characteristic multinucleated osteoclast-like giant cells. Some of the histiocytes may have pale foamy cytoplasm.

35. Ainhum • Linear constriction occurs around the toes (esp 5th at PIP) eventually resulting spontaneous amputation in 5-10 years. Begins as grove on flexor surface and joins over time. • African Men. • Etiology: Unknown. (Trauma?) • TX: Surgery sometimes helpful. Intralesional steroids. • Pseudo-Ainhum: Hereditary and nonhereditary diseases associated with annular constriction.

36. Ainhum

37. Ainhum

38. Connective Tissue Nevi • Multiple or solitary plaques 1-15 cm in diameter, yellow to light orange with a shagreen leather like surface texture. • Predilection for lumbosacral area. • Acquired type: eruptive collagenoma, isolated collagenoma and isolated elastoma • Congenital Types: • 1.) Buschke – Ollendorf: AD. Widespread asymmetrically distributed plaques. Osteopoikilosis of long bones is diagnostic.

39. Connective Tissue Nevi (Continued) • 2.) Familial cutaneous collagenomas: Numerous symmetrical asymptomatic nodules on back. Onset in teens. MEN-I assoc. • In tuberous sclerosis, connective tissue nevi are shagreen patches. • ½ of T.S. cases are new mutations so any pt with connective tissue nevi should be evaluated for tuberous sclerosis.

40. Connective Tissue Nevi

41. Angiofibromas Fibrous and vascular proliferation of upper dermis Common types: • Fibrous papule of the nose – 3-6 mm diameter, dome shaped sessile papule with white to reddish color. Usually solitary. • Pearly Penile Papule – Pearly white papules appearing on the coronal margin and sometimes on penile shaft. No TX required • Multiple hereditary forms - adenoma sebaceum (Tuberous Sclerosis) and in MEN-I

42. Angiofibromas Fibrous papule of the nose Pearly Penile Papule

43. Acral Fibrokeratoma • Pink, hyperkeratotic, hornlike projection on fingers (most common) toes or palms. Emerges from a collarette of scale. • Average age 40. • TX: excision. Laser ablation.

44. Acral Fibrokeratoma

45. Subungal Exostosis • Solitary, fibrous and bony nodule protruding from the distal edge of the nail, most commonly of the great toe. • Begins as a pink papule which destroys overlying nail and grows to a maximum diameter of 8 mm. • Pressure causes great pain. • X-ray is diagnostic • TX: excision and curettage.

46. Subungal Exostosis

47. Chondrodermatits Nodularis Chronica Helicis • Small, tender, inflammatory nodule with gently sloping sides located on the outer helix. Common in older men. No malignant potential. • Often hx of chronic trauma. Lesions are very painful. Pt often complains of pain when sleeps on lesion. • Histology: Degeneration of collagen with acanthosis and hyperkeratosis and thinning of epidermis. • TX: Excision. Often a spicule of cartilage is discovered. (Transdermal elimination).

48. Chondrodermatits Nodularis Chronica Helicis

49. Acrocordon • Flesh colored, pigmented sessile or pedunculated papillomas. • Areas: eyelids, axilla, neck, groin. • 60 % incidence by the age of 69. • Treatment: excision.

50. Dermatofibroma • Lesion which grows slowly to a firm, 4-20 mm diameter papule or nodule, yellow or reddish brown in color and then stops expanding. Sharply demarcated. Chiefly located on lower extremities. • ‘Dimple sign’. Seldom seen in children. May grow to 5 cm in size. • Etiology: Trauma, bites ? • Histology: Dermal mass or whorled fibrous tissue. Numerous cells with large nuclei and spindle cells. Some very atypical cells referred to as ‘Monster cells’. • TX: Reassurance. Progressive enlargement warrants excision.