Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis

Abstract

A case of retroperitoneal lymphangioleiomyomatosis (LAM) arising from endosalpingiosis is described. A 25-year-old woman with no history of tuberous sclerosis or hormonal therapy presented with a painless, palpable abdominal mass. Computed tomographic and magnetic resonance imaging studies of the abdomen demonstrated a 4 cm cystic mass in the retroperitoneum. Macroscopically, the excised retroperitoneal cyst was multilocular and measured 4.0 x 3.5 x 3.5 cm. Histologically, the lesion demonstrated three components. The first comprised multiple cysts or glands lined by columnar epithelial cells with cilia. The second component was a condensation of small stromal cells immediately subjacent to the cystic epithelium or glands. The third component was a thick exterior wall composed of plump spindle cells with clear to palely eosinophilic cytoplasm in a fascicular pattern, and slit-like vascular spaces, resembling LAM. Immunohistochemically, the epithelium and glands were positive for cytokeratin 7. The stromal cells were positive for vimentin and CD10. The cells of the LMA-like component showed positive staining for HMB45, alpha-smooth muscle actin, muscle actin and h-caldesmon. The lesion, LAM arising from endosalpingiosis, represents a distinctive pathologic entity that should be recognized and studied further. This type of lesion should be included in the differential diagnosis of retroperitoneal cystic lesions.

Extrapulmonary Lymphangioleiomyomatosis in Pelvic and Paraaortic Lymph Nodes Associated With Uterine Cancer

“…LAM also arises in extrapulmonary organs such as lymph nodes, retroperitoneum, and mediastinum. Extrapulmonary LAM is often associated with pulmonary involvement, and extrapulmonary LAM without pulmonary involvement described in this study is extremely rare (2,3,8,9).…”

Lymphangioleiomyomatosis with Florid Endosalpingiosis

“…Lymphangioleiomyomatosis with endosalpingiosis has been reported to involve the retroperitoneum [4,5]. In our case, both the retroperitoneum and left broad ligament were involved.…”

“…In our patient, the elongated mass along the left broad ligament remained static in size on CT follow-up at a 1.5-year interval, and the patient's symptoms remained stable during that time. Regarding the prognosis of this disease entity, the lesion would be expected to have a benign clinical course because retroperitoneal lymphangioleiomyomatosis rarely shows an aggressive course [4,5].…”

Association of retroperitoneal lymphangioleiomyomatosis with endosalpingiosis: a case report

“…Prevalence of extrapulmonary LAM with endosalpingiosis is unknown and there are only three cases reported in the literature [1,6]. Matsui et al [1] described two women, 36 and 50-year-old, respectively, without previous pulmonary symptoms.…”

“…Fukunaga et al . [6] reported a third case of extrapulmonary LAM associated with endosalpingiosis in a 25-year-old woman. This patient did not have any pulmonary affectation, as the aforementioned case.…”