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

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