Department of Medical, Oral and Biotechnological Sciences, University G. D'Annunzio, Chieti-Pescara, Via dei Vestini, 66100, Chieti, Italy.
Surgical Pathology Unit, Santissima Annunziata Hospital, Chieti, Italy.
BMC Surg. 2020 Nov 25;20(1):297. doi: 10.1186/s12893-020-00951-0.
Leiomyosarcoma usually develops in the myometrium and is characterized by a high recurrence rate, frequent hematogenous dissemination, and poor prognosis. Metastasis is usually to lungs, liver, and bone, and occasionally to the brain, but seldom to the head and neck region. Primary leiomyosarcoma very rarely arises in the broad ligament.
A 54-year old woman presented to the otolaryngology department with a mass in the right posterior region of the neck 4 years after surgery for a primary leiomyosarcoma of the right broad ligament. The neck mass was removed and found to be a metastatic leiomyosarcoma. Leiomyosarcoma localizations in lungs and liver were absent. Morphological examination showed both the primary and the secondary leiomyosarcomas to have features of low-grade tumors. One year after excision of the neck mass, the patient presented with tachycardia. Echocardiography detected two intracardiac nodules suggestive of metastatic tumors. Chemotherapy was administered; the disease has been stable since then.
We report the first case of broad ligament leiomyosarcoma with the neck subcutaneous region being the first site of secondary involvement. We speculate that the Batson venous plexus might have been the pathway of dissemination.
平滑肌肉瘤通常发生在子宫肌层，其特点是复发率高、频繁血行播散和预后不良。转移通常发生在肺部、肝脏和骨骼，偶尔发生在脑部，但很少发生在头颈部。原发性平滑肌肉瘤极罕见于阔韧带。
一名 54 岁女性在因右侧阔韧带原发性平滑肌肉瘤手术后 4 年因颈部右侧后区肿块就诊于耳鼻喉科。切除颈部肿块后发现为转移性平滑肌肉瘤。肺部和肝脏均无平滑肌肉瘤定位。形态学检查显示原发性和继发性平滑肌肉瘤均具有低度肿瘤的特征。切除颈部肿块 1 年后，患者出现心动过速。超声心动图检测到两个提示转移性肿瘤的心内结节。给予化疗，此后病情稳定。
我们报告首例阔韧带平滑肌肉瘤，其颈部皮下组织为继发累及的首发部位。我们推测 Batson 静脉丛可能是播散途径。
