Malignant clear-cell myomelanocytic tumor of broad ligament—a case report

Abstract

Clear-cell myomelanocytic tumors (CCMT) of the perivascular epithelioid cell tumor (PEComa) family have been recently reported. We report a case involving a 12-year-old girl. The tumor (9 x 7.5 x 7 cm) was a firm, tan-gray mass with heavily dark pigmentation, massive hemorrhage, and necrosis, and was located in the right broad ligament attached to the right ovary. Histologically, the tumor was composed of polygonal cells exhibiting diffuse hemorrhage, multifocal necroses, and vascular invasion. Most of the tumor cells contained melanin pigments with Fontana-Masson positivity and ultrastructurally suspicious, membrane-bound premelanosomes. Immunohistochemical staining was positive against HMB-45 and focally positive for smooth muscle actin. The tumor recurred in the form of multiple conglomerated masses of the right iliac fossa, with the greatest measuring up to 3.8 cm in dimension, within 1 year. Most CCMT are believed to originate from falciform ligament/ligamentum teres. To the best of our knowledge, this is the second report of a CCMT arising in the broad ligament with typical morphology and contributory ancillary results. Further study for proper subclassification of the PEComa family should be validated, not by anatomic site but by clinical behavior.

Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

“…This is further supported by the different anatomical distribution, being the genital areas prevalent in women independent of age and the involvement of the intestinal wall quite frequent in men (45%). In children, [6,[14][15][16][17][18][19][21][22][23]25,26,[28][29][30][32][33][34][35] 2 (focal)/21 MART-1 [14,18,33,36] 2/4 Melan-A [6,15,16,18,[20][21][22][23]25,26,28] 6/12 MiTF 22,23,28] 0/3 TFE3 [22,23,25,28] 5/5 Caldesmon [14,15,33] 0/3 Calponin [14,15,36] 3/3 SMA [14-18,20,21,23-26, 28,29,33,34,36] 7/18 Vimentin [17,…”

A case of pararenal PEComa with extensive bone metaplasia

“…In sum, this unusual case belongs to the PEComa family, a recently defined group of tumours originated from perivascular epithelioid (PEC) cells [1,2] at multiple sites, encompassing angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumour and rare tumours in other locations. They have a wide range of clinical, pathological and immunohistochemical features: clearly malignant [3,5], some of uncertain malignant potential and a few benign. This case would fulfil the criteria of the benign group: small (<5 cm), bland cellularity, no mitosisnecrosis-vascular invasion [1]; it had wide hyalinised areas and frequent calcification and osseous metaplasia, an unusual feature (which made it more evident in the CT scan).…”

Broad ligament perivascular epithelioid cell tumor (pecoma) of uncertain malignant potential

“…PEComas are treated by excision of the mass, salpingooophorectomy or total abdominal hysterectomy with bilateral salpingo-oopherectomy (3,6,8). Sirolimus, an immunosuppressive agent, inhibits the activity of mTOR and is useful in the treatment of PEComa (10).…”

“…PEComas arising in the broad ligament are extremely rare. Only seven cases have been reported in the literature (3)(4)(5)(6)(7)(8)(9). We report a case of PEComa involving the broad ligament in a young lady encasing the ureter resulting in obstructive uropathy.…”