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Uterine Corpus Cancer
Published in Dongyou Liu, Tumors and Cancers, 2017
Tumors of the uterine corpus consist of (1) epithelial tumors and related lesions (endometrial carcinoma [endometrioid adenocarcinoma—variant with squamous differentiation, villoglandular variant, secretory variant, ciliated cell variant; mucinous adenocarcinoma; serous adenocarcinoma; clear cell adenocarcinoma; mixed cell adenocarcinoma; squamous cell carcinoma; transitional cell carcinoma; small cell carcinoma; undifferentiated carcinoma], endometrial hyperplasia [nonatypical hyperplasia—simple or complex or adenomatous, atypical hyperplasia—simple or complex], endometrial polyp, tamoxifen-related lesions), (2) mesenchymal tumors (endometrial stromal and related tumors [endometrial stromal sarcoma—low grade, endometrial stromal nodule, undifferentiated endometrial sarcoma], smooth muscle tumors [leiomyosarcoma—epithelioid variant or myxoid variant; smooth muscle tumor of uncertain malignant potential; leiomyoma NOS—histological variants, mitotically active variant, cellular variant; hemorrhagic cellular variant, epithelioid variant, myxoid, atypical variant, lipoleiomyoma variant; growth pattern variants—diffuse leiomyomatosis, dissecting leiomyoma, intravenous leiomyomatosis, metastasizing leiomyoma], miscellaneous mesenchymal tumors [mixed endometrial stromal and smooth muscle tumor, perivascular epithelioid cell tumor, adenomatoid tumor, other malignant mesenchymal tumors, other benign mesenchymal tumors]), (3) mixed epithelial and mesenchymal tumors (carcinosarcoma [malignant Müllerian mixed tumor, metaplastic carcinoma], adenosarcoma, carcinofibroma, adenofibroma, adenomyoma—atypical polypoid variant), (4) gestational trophoblastic disease (trophoblastic neoplasms [choriocarcinoma, placental site trophoblastic tumor, epithelioid trophoblastic tumor]; molar pregnancies [hydatidiform mole—complete, partial, invasive, metastatic]; nonneoplastic, nonmolar trophoblastic lesions [placental site nodule and plaque, exaggerated placental site]), (5) miscellaneous tumors (sex cord–like tumors, neuroectodermal tumors, melanotic paraganglioma, tumors of germ cell type), (6) lymphoid and hematopoietic tumors (malignant lymphoma, leukemia), and (7) secondary tumors [1].
Preoperative adrenal artery embolization followed by surgical excision of giant hypervascular adrenal masses: report of three cases
Published in Acta Chirurgica Belgica, 2018
Ismail Cem Sormaz, Fatih Tunca, Arzu Poyanlı, Yasemin Giles Şenyürek
The patient underwent DSA 24 h prior to the intervention. Angiography images showed that the right adrenal mass was predominantly fed by inferior phrenic artery. The inferior phrenic artery was selectively catheterized with 2.8 French microcatheter (Figure 3(a)) and embolized with polyzene-F hydrogel microspheres (six boxes of 1300 μm and one box of 1100 μm). At the end of the procedure, test imaging showed almost complete disappearance of tumor blush (Figure 3(b)). The patient was closely monitored at the ICU during the subsequent 24 h and had no symptoms associated with arterial embolization. The patient underwent surgical exploration via extended right subcostal incision and right adrenalectomy was performed. During the operation, we observed marked decrease in the hypervascularity of the tumor. The size of the vessels, especially veins, surrounding the adrenal tumor was found to be decreased when compared to preoperative imaging findings. No major blood loss occurred during the operation. The postoperative period was uneventful and the patient was discharged on the 5th postoperative day. The final histopathologic examination revealed perivascular epithelioid cell tumor (PEComa) and the largest diameter of the tumor was measured as 18 cm.
PEComa of the talus: a unique case of a soft tissue tumor within bone
Published in Acta Chirurgica Belgica, 2019
Michael Gebhart, Andrei Coltofeanu
The World Health Organization coined the term of perivascular epithelioid cell tumor (PEComa) as ‘a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells’ [1]. The perivascular epithelioid cell (PEC) is a cell type that is present in a group of tumors including angiomyolipoma (AML), clear cell sugar tumor (CCST), lymphangioleiomyomatosis (LAM) and a number of rare unusual visceral, intra-abdominal, soft tissue and even bone tumors which have been described under a variety of names [1]. In 1992, Bonetti et al. observed a link between this group of tumors (AML, CCST and LAM) with tuberous sclerosis complex (TSC), an autosomal dominant genetic disease and he was the first one to describe a family of neoplasms that has a unique type of cells, which had ‘immunoreaction with melanocytic markers and exhibited an epithelioid appearance, a clear cell acidophilic cytoplasm, and a perivascular distribution’ [2]. In 1996, the term of ‘PEComa’ was used for the first time to describe the family of lesions due to a ‘pure’ proliferation of PECs [1]. PEComas show a positive immunostaining for both myogenic and melanocytic markers such as HMB45, MelanA, tyrosinase, microphthalmia transcription factor, actin and desmin [2]. Histologically, in these tumors, we can find both epithelioid and spindle cells arranged tightly around thin vessels or in radiating pattern. Respecting these elaborate ‘architecture’, the cells are forming a well vascularized ‘nest’ [1]. As only few cases of malignant PEComa have been reported, with a relative short follow-up period, we do not have very strong criteria for malignancy. However, few criteria were reported for malignancy such as size of tumor >5 cm, mitotic activity >1/50 high-power fields, cell necrosis, nuclear pleomorphism and infiltrative growth pattern [3]. PEComas have been reported in different organs and are considered quite ubiquitous tumors. Although this tumor is very rare, most of them arise within soft tissues, visceral organs and skin [3]. Only three well documented cases of PEComas are described as primary bone localization [4–6].