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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.

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