Uterine Tumors with Neuroectodermal Differentiation. A Report of 4 Cases

Abstract

We report four cases of uterine tumors with neuroectodermal differentiation. One tumor had neuroectodermal component only; in the three other tumors, the neuroectodermal component was admixed with another component, namely rhabdomyosarcoma (1 case), and endometrioid adenocarcinoma (2 cases). Histologically, the neuroectodermal component consisted of small to medium sized cells arranged in diffuse sheets. The tumor cells had round nuclei with stippled to coarsely granular chromatin, mostly with non-prominent nucleoli, and scant eosinophilic or amphophilic cytoplasm. Immunohistochemically, 4/4 tumors showed expression of vimentin, synaptophysin and CD56; 3/4 tumors were CD99 and NSE positive; 2/4 tumors showed focal expression of S-100 protein; and 1/4 tumors had focal dot-like cytoplasmic positivity for cytokeratin AE1/AE3. FLI-1 was negative in all cases. FISH examination was performed and none of the tumors showed rearrangement of EWSR1 gene. Uterine tumors with neuroectodermal differentiation are rare; to the best of our knowledge only 44 cases have been reported in the literature to date, referred to as Ewing sarcoma, peripheral PNET (pPNET), PNET (not otherwise specified) and uterine tumors with neuroendocrine differentiation.

Uterine adenosarcoma admixed with a primitive neuroectodermal tumour (PNET): a case report and review of the literature

“…The present review of the literature yielded 30 cases of uterine PNET combined with other malignancies ( Table 2). Other than adenosarcoma (two cases) [4,8], PNET have been found admixed with endometrioid endometrial carcinoma (15 cases) [4,[9][10][11][12], malignant mixed Müllerian tumour (MMMT) (two cases) [4], high grade serous endometrial carcinoma (five cases) [12], rhabdomyosarcoma (four cases) [4,9,[13][14], and endometrial stromal sarcoma (two cases) [4,10]. A Müllerian derivation for at least some of these uterine PNET has been advocated, due to the prevalence of cases associated with a Müllerian neoplasm (Table 2).…”

“…Gersell et al and Fukunaga et al cases contained mature neural elements such as ganglion cells, or glia, and expressed GFAP, which is not a feature of PNET [15,16]. Rare cases of combined rhabdomyosarcoma and PNET have been also described [4,9,13,14] ( Table 2).…”

Adjuvant chemotherapy for a primitive neuroectodermal tumor of the uterine corpus: A case report and literature review

“…Reports with incomplete information about chemotherapy were also excluded. Of a total of 178 papers identified from the search terms in the MEDLINE database, 49 cases fulfilled the mentioned criteria (Table ). We analyzed the choice of chemotherapy regimen in relation to the patients’ ages and divided patients into the following groups: adolescence (<20 years), reproductive (20–40 years), premenopausal (40–60 years) and elderly (>60 years).…”

Heterologous and Rare Homologous Sarcomas of the Uterine Corpus

“…1B), as are numerous mitotic figures, including atypical forms. Combining data from the series of Dundr et al 55 and Euscher et al 57 yields the immunophenotypic profile outlined in Table 2. The differential diagnosis for uterine ES/PNET is extensive, and is beyond the scope of this contribution.…”

“…67,68 In uterine ES/PNET, however, rearrangements in the EWSR1 gene have only been found in 4 (20%) of the 20 tested cases. 41,48,55,57,58 In the author's opinion, the tumors that have been referred to as ES/PNET of the uterus, although morphologically similar, are histogenetically heterogeneous. In a small subset, probably approximately 20%, they are akin to typical, peripheral, osseous-type ES/PNET, and this is the group displaying EWSR1 rearrangements.…”