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
- Differentiate from leiomyosarcoma
- 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
- Lesions may be calcified
- 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
- 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
- Masson trichrome and PTAH (phosphotungstic acid-hematoxylin) demonstrates myofibrils
- EM: myofilaments, surface pinocytotic vesicles, basal laminae
- 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
- 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
- Distinct from the uterus
- Occasionally multiple
- Usually estrogen +, always progesterone +
- 1-10 mitoses/50 HPF
- LR with + margin has been reported
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