Extrapulmonary lymphangioleiomyoma presenting as a mesenteric cystic mass

Abstract

Lymphangioleiomyoma (LAM) is a rare benign tumor characterized by a disorganized smooth muscle cell proliferation within the walls of pulmonary, mediastinal, and retroperitoneal lymphatic vessels. The mesenteric LAM is a quite unusual presentation. A 47-year-old woman was admitted to our hospital with a 7-year history of abdominal mass. A computed tomography showed a well marginated mass with soft tissue attenuation. Laparoscopic excision was performed. The specimen revealed a well circumscribed dark cystic mass. The cut surface showed a thin walled cystic tumor with haphazardly distributed trabeculated septa. Microscopically, the tumor showed variable sized ectatic spaces lined by flattened endothelial cells and surrounded by epithelioid or spindle-shaped cells. The lining cells were positive for D2-40, CD31, and CD34. The surrounding cells were positive for alpha-smooth muscle actin and human melanoma black-45. The final diagnosis was a solitary LAM arising in the mesentery. The patient showed an uneventful postoperative course for 10 months.

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

“…Lymphangioleiomyoma is most commonly localized in the retroperitoneal space and the uterus and in surrounding lymph nodes [6,24,27]. Moreover, E-LAM foci developing in the mediastinum [36], supraclavicular lymph nodes [37,38], liver [39], walls of the small and large intestine [40,41], pancreas [26,42], mesentery [43] have been described. E-LAM masses can reach very large sizes -cases of tumors with the greatest length 15-22 cm have been described [18,[44][45][46] -and can extend along lymph vessels -coming from the retroperitoneal space to the chest [19], and further even along the neck along with the sternocleidomastoid muscle [47].…”

“…They also found the presence of T lymphocytes showing the expression of programmed death-ligand 1 (PD-1) receptor, infiltrating LAM foci [66]. In the case of lymphangioleiomyoma, LAM cells show the expression of a similar marker profile: HMB45 -particularly in epithelial cells [6], Melan A, calponin, SMA, desmin, nuclear expression of ER and PR [6,24,46,50]; moreover expression of b-catenin [35], E-cadherin [67], MiiTF [25] and in the lymph vessel endothelium: CD34, podoplanin, CD31, and VEGFr-3 [21,24,26,43].…”

“…In a retrospective analysis by Matsui et al (2001) among 17 patients with E-LAM, only one died because of an aggressive course of a simultaneously occurring pulmonary LAM form, all the others were alive at the moment of publishing the results with an average observation time of 5.5 years [6]. Radical resection allows long-term control of the disease [16,24,39,40,42,50], also when laparoscopic techniques are used [43,113]. There are reports in the literature about local recurrence after E-LAM resection [37,114], which can, however, be treated with good effects by a repeated resection [115].…”