Kilpatrick S E, Mentzel T, Fletcher C D
Department of Pathology, North Carolina Baptist Hospital, Bowman-Gray School of Medicine, Winston-Salem.
Am J Surg Pathol. 1994 Jun;18(6):576-82. doi: 10.1097/00000478-199406000-00003.
Leiomyomas of deep soft tissue are extremely rare and, to our knowledge, have never been adequately characterized either histologically or clinically. We report 11 such cases, most often arising in deep subcutis or skeletal muscle. The lesions were equally distributed among males (six cases) and females (five cases); age at presentation ranged from 14 to 62 years (mean, 37 years; median, 35 years). The extremities represented the single most common site (five cases). Characteristic morphologic features included well-defined circumscription with a fibrous pseudocapsule, myxohyaline stromal degeneration, and intersecting fascicles of spindle cells with mostly uniform, round-ended, elongated nuclei and tapering, eosinophilic cytoplasm. Dystrophic calcification was identified in four cases; degenerative nuclear changes, similar to those in ancient schwannoma, were present in three cases. Two neoplasms showed multiple necrobiotic granuloma-like nodules resembling giant rosettes. Mitotic figures were found in only four cases and were scarce, never exceeding one mitotic figure per 50 high-power fields. Immunohistochemically, all cases showed desmin positivity. Clinical follow-up (median, 4.5 years), available for nine cases, revealed no evidence of local recurrence or distant metastasis. As with all smooth-muscle cell neoplasms, meticulous attention to the presence of significant mitotic activity and suppurative tumor necrosis is essential because they suggest potential malignant behavior.
深部软组织平滑肌瘤极为罕见,据我们所知,其在组织学或临床上均未得到充分的特征描述。我们报告了11例此类病例,大多发生于深部皮下组织或骨骼肌。这些病变在男性(6例)和女性(5例)中分布均等;发病年龄为14至62岁(平均37岁;中位数35岁)。四肢是最常见的单一部位(5例)。特征性形态学特征包括边界清晰,有纤维性假包膜,黏液透明样基质变性,以及梭形细胞的交叉束状排列,细胞核大多均匀、圆形、细长,细胞质嗜酸性且逐渐变细。4例中发现营养不良性钙化;3例出现类似于陈旧性神经鞘瘤的退行性核改变。2例肿瘤显示多个类似巨大玫瑰花结的坏死性肉芽肿样结节。仅4例发现有丝分裂象,且数量稀少,每50个高倍视野中丝分裂象从不超过1个。免疫组化显示,所有病例结蛋白均呈阳性。9例患者有临床随访(中位数4.5年),未发现局部复发或远处转移的证据。与所有平滑肌细胞肿瘤一样,必须密切关注是否存在显著的有丝分裂活性和化脓性肿瘤坏死,因为它们提示可能存在恶性行为。