Leiomyoma

DEFINITION AND PATHOGENESIS

• Cutaneous leiomyoma (leiomyoma cutis)
• Arises from the piloerector muscles of skin (arrectores pilorum)
• Angiomyoma (vascular leiomyoma, angioleiomyoma)
• Solitary subcutaneous lesion with numerous thick-walled vessels; arise from vessel walls
• Leiomyoma of deep soft tissue
• A rare larger deep seated smooth muscle lesion of extremities, abdomen or retroperitoneum

IMPORTANCE

• Differentiate from leiomyosarcoma

CLINICAL FEATURES

• Cutaneous lesions
• Often multifocal, small discrete papules up to 1-2cm, may follow dermatomal distribution
• Most often involve extensor surfaces
• May be associated with pain and tenderness, often paroxysmal
• Usually 15-25 years of age, occasionally younger, even at birth
• May be familial, ? AD
• Multiple lesions associated with:
  • Dermatitis herpetiformis; HLA-B8
  • Premature uterine leiomyomas

• • ? erythropoietin
  • MEA Type I
  • Have been associated with HIV
  • Angiomyoma (vascular leiomyoma, angioleiomyoma)
• Patients generally 30-60 years of age
• Lower leg most often affected (67%), UE in 22%
• M:F = 1:1-2
• 50% with pain ? exacerbation with pressure, temperature change, pregnancy, menses
• Intraneural location in the median nerve has been reported, dispersing fascicles around the lesion
• Deep soft tissue lesions

RADIOLOGIC FEATURES

• Lesions may be calcified
• MRI
  • hyperintense to skeletal muscle on T1 and hyper- and isointense areas on T2 weighted images
  • Signal voids within the mass in angiomyomas may represent vessels

GROSS PATHOLOGY

• Cutaneous lesions poorly defined, blending in with dermal collagen
• Angiomyomas are subcutaneous circumbscribed, grayish-white glistening nodules, rarely with Ca++, visibly contract when manipulated, most <2cm in diameter
• Deep seated lesions are grayish-white small circumscribed tumors which may be gelatinous in nature

HISTOLOGIC FEATURES

• Masson trichrome and PTAH (phosphotungstic acid-hematoxylin) demonstrates myofibrils
• EM: myofilaments, surface pinocytotic vesicles, basal laminae
• Angiomyoma
  • Nodule of smooth muscle fibers swirling away from thick-walled vessels, ? areas of hyalinization, calcification; nerve fibers within capsule and in the lesion
• Deep seated lesions
• Intersecting muscle cells ? degenerative changes
• May have nuclear palisading and perinuclear vaculolization
• Cytoplasmic glycogen and fuchsinophilia, and longitudinal striations
• Rarely with "clear cell cytoplasmic changes"
• + desmin in benign smooth muscle tumors
• Regressive changes: fibrosis, calcification, and rarely ossification in large lesions
• Focal calcification may look like psammoma bodies
• Rarely with nuclear atypia but not mitoses (pleomorphic leiomyoma

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

• Hamartomatous deposits of smooth muscle
• Dermatofibroma (cutaneous fibrous histiocytoma)
• Nodular fasciitis or myoma when deep seated lesions undergo myxoid changes
• Clear cell carcinoma or melanoma when with clear cell changes
• Leiomyosarcoma in deep seated lesions

DISEASE COURSE AND TREATMENT

• Cutaneous lesions
  • 50% develop recurrences or new cutaneous lesions
• Nitroglycerin and phenoxybenzamine may be helpful for symptomatic relief
• Angiomyomas
  • Simple excision resolves pain, rarely recur
  • Malignant transformation has been reported in a LR in a digital location
• Deep leiomyomas
  • Require complete excision
  • Too rare to determine prognosis
• Angioleiomyoma has been reported occurring within bone
• Beware of sampling error missing dx of leiomyosarcoma
• Two subcategories:
  • Leiomyomas of somatic soft tissue
    • Estrogen and progesterone receptor –
    • No LR or metastasis
    • 1-4 mitoses/50 HPF
  • Retroperitoneal-abdominal leiomyomas
    • F>>M
    • Distinct from the uterus
    • Occasionally multiple
    • Usually estrogen +, always progesterone +
    • 1-10 mitoses/50 HPF
    • LR with + margin has been reported

SPECIAL CONSIDERATION

• CUTANEOUS LEIOMYOMA

• MYOLIPOMA, LIPOLEIOMYOMA

• ANGIOLEIOMYOMA

REFERENCES

Billings SD, Folpe AL, Weiss SW: Do leiomyomas of deep soft tissue exist? An analysis of highly differentiated smooth muscle tumors of deep soft tissue supporting two distinct subtypes. Am J Surg Pathol 25:1134-42,2001.


Bogumill GP, Sullivan DJ, Baker GI: Tumors of the hand 108:214-22,1975.


Botte MJ, Silver MA: Leiomyoma of a digital artery. Clin Orthop 260:259-62,1990.


Bulmer JH: Smooth muscle tumors of the limbs. J Bone Joint Surg 49B:52-8,1967.


Callé SC, Eaton RG, Littler JW: Vascular leiomyomas in the hand. J Hand Surg 19A:281-6,1994.


Cooperman B, McAllister FF, Smith FM: Recurrent calcified leiomyoma of the pelvis and gluteal regions complicated by pregnancy. J Bone Joint Surg 40A:1149-53,1958.


Dockery GL, Wendel RE: Benign vascular leiomyoma-angioma of the digit: a case report. J Am Podiatry Assoc 69:438-9,1979.


Drew EJ: Large leiomyoma of upper extremity. Am J Surg 112:938-40,1966.


Duinslaeger L, Vierendeels T, Wylock P: Vascular leiomyoma in the hand. J Hand Surg 12A:624-7,1987.


Enzinger FM, Weiss SW: Benign Tumors of Smooth Muscle. In Soft Tissue Tumors FM Enzinger and SW Weiss (Eds), C.V. Mosby Co., St. Louis (3rd ed) 1995, pp 467-90.


Fernandez-Pugnaire MA, Delgado-Florencio V: Familial multiple cutaneous leiomyomas. Dermatology 191:295-8,1995.


Freedman AM, Meland NB: Angioleiomyoma of the extremities. Report of a case and review of the Mayo Clinic experience. Plastic Reconstr Surg 83:328-331,1989.


Galinski AW, Aune CJ: Benign leiomyoma of the foot: a case report. Clin Podiatr Med Surg 5:359-62,1988.


Giannakopoulos PN, Sotereanos DG, Tomaino MM, Goodman MA, Herndon JH: Benign and malignant muscle tumors in the hand and forearm. Hand Clin 11:191-201,1995.


Goodman AH, Briggs RC: Deep leiomyoma of an extremity. J Bone Joint Surg 47A:529-32,1965.


Hachisuga T, Hashimoto H, Enjoji M: Angioleiomyoma: a clinicopathologic reappraisal of 562 cases. Cancer 54:126-30,1984.


Hanft JR, Carbonell JA, Do HQ: Angioleiomyoma of the lower extremity. J Am Podiatr Med Asso 87:388-91,1997.


Hasegawa T, Seki K, Yang P, Hirose T, Hizawa K: Mechanism of pain and cytoskeletal properties in angioleiomyoma: an immunohistochemical study. Pathol Int 44:66-72,1994.


Herren DB, Zimmermann A, Büchler U: Vascular leiomyoma in an index finger undergoing malignant transformation. J Hand Surg 20B:484-7,1995.


Herrlin K, Willen H, Rydholm A: Deep seated soft tissue leiomyomas: report of four cases. Skeletal Radiol 19:363-5,1990.


Hwang JW, Ahn JM, Kang HS, Suh JS, Kim SM, Seo JW: Vascular leiomyoma of an extremity: MR imaging – pathology correlation. AJR 171:981-5,1998.


Kataoka M, Yano H, Fukunaga T, Masumi S: Giant vascular leiomyoma in the hand. Scand J Plast Reconstr Surg Hand Surg 31:91-3,1997.


Kilpatrick SE, Mentzel T, Fletcher CDM: Leiomyoma of deep soft tissue: clinicopathologic analysis of a series. Am J Surg Pathol 18:576-82,1994.


Lawson GM, Salter DM, Hooper G: Angioleiomyomas of the hand. J Hand Surg 20B:479-83,1995.


Ledesma-Medina J, Oh KS, Girdany BR: Calcification in childhood leiomyoma. Radiology 135:339-41,1980.


López-Barea F, Rodrígues-Peralto JL, Burgos E, Gonzalez-Lopez J: Calcified leiomyoma of deep soft tissue: report of a case in childhood. Virchows Arch 425:217-20,1994.


Meis JM, Enzinger FM: Myolipoma of soft tissue. Am J Surg Pathol 15:121-5,1991.


Myhre Jensen O: A consecutive 7-year series of 1331 benign soft tissue tumours. Acta Orthop Scand 52:287-93,1981.


Neviaser RJ, Newman W: Dermal angiomyoma of the upper extremity. J Hand Surg 2:271-4,1977.


Piers W, Terrono AL, Hayek J, Millender LH: Angiomyoma (vascular leiomyoma) of the median nerve. J Hand Surg 21A:285-6,1996.


Robinson SC, Kalish RJ: Leiomyoma in the hand. A case report. Clin Orthop 255:121-3,1990.


Ross LS, Eckstein MR, Hirschorn R, Khapra AH, Shulman S, Hochstim RJ: Calcified leiomyoma. An unusual cause of large soft-tissue calcification of calf in childhood. NY State J Med 83:747-9,1983.


Shapiro JD, Froimson AI: Angiomyoma in the hand. Orthopedics 11:1709-10,1988.


Sonobe H, Ohtsuki Y, Mizobuchi H, Toda M, Shimizu K: An angiomyoma with t(X;10)(q22;q23.2). Cancer Genet Cytogenet 90:54-6,1996.


Spinosa FA: Leiomyoma of the foot. J Foot Surg 24:68-70,1985.


Stout AP: Solitary cutaneous and subcutaneous leiomyoma. Am J Cancer 29:435-69,1937.


Sweeney J, Keating SE: Angioleiomyoma. J Foot Surg 22:21-2,1983.


Tomoda K, Iyama K: A case of intraosseous angioleiomyoma. Acta Orthop Scand 63:568-70,1992.


Trail IA, Noble J, Freemont A: Intraneural leiomyoma: a brief report. J Bone Joint Surg 71B:140,1989.


Watson GM, Saifuddin A, Sandison A: Deep soft tissue leiomyoma of the thigh. Skeletal Radiol 28:411-4,1999.