Retroperitoneal Lymphangioleiomyoma Mimicking Ovarian Tumor Emerging After Tamoxifen Therapy

Abstract

Isolated retroperitoneal lymphangioleiomyoma is rare and difficult to detect in the absence of pulmonary lymphangioleiomyomatosis. We speculate that tamoxifen treatment may play a role in the development of this benign tumor.

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

“…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].…”

“…These cells form nests -resembling the so-called "zellballen" described in phaeochromocytomas [26], separated by slot-like vessels with thin walls lined with endothelial cells [50], which may undergo hyalinization [21,24,26]. Typically necrosis and blood extravasation to the tumor are not observed [6,21,26,46]. In most cases the mitotic activity is low [6,21], in some cases, up to 4 figures for 10 visual fields were observed [24,37].…”

“…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].…”

“…The symptoms of lymphangioleiomyoma presence occur in about 55-60% patients [6,18] and the most commonly are: pain in the vicinity of the tumor [26,29,46] and bleeding from the reproductive system in the case of LAM localized in the uterus [24,48,49]. Symptoms associated with the pressure of the tumor mass on neighboring organs are also frequently observed: dyspepsia, bloating and symptoms of obstruction of the alimentary tract [46], hydronephrosis or edema and paresthesia of the lower extremities [46,50]. Lymphangioleiomyoma localized in the mediastinum may cause Horner syndrome, lymph exudates to the pleural cavity and heart rhythm perturbations [6,18].…”

“…Lymphangioleiomyoma in computer tomography most commonly takes the form of well-defined, solid-cystic lesions, with walls of different thickness and numerous septs [26,27,46]. Less commonly a solid or only cyst-like character is observed [27].…”