Mucinous Carcinoid as an Unusual Manifestation of Endodermal Differentiation in Ovarian Yolk Sac Tumors

Abstract

We present, for the first time, two yolk sac tumors (YST) in women 37 and 18 years of age, one with a typical parietovisceral pattern and the other with a glandular pattern, which were associated with extensive areas of mucinous carcinoid (MC). The tumor in the first case had numerous nodules of tubulopapillary YST that merged with well-differentiated MC. This patient responded well to chemotherapy. The tumor in the second case consisted of an AFP-positive glandular YST, with a glandulopapillary pattern closely resembling fetal lung type adenocarcinoma, coexisting with an AFP-negative, cytokeratin 20-positive, atypical MC; transitional areas between the two components were also seen. In the material from the recurrences and metastases; however, no YST was present, the atypical MC having become the predominant component including areas that had become carcinomatous. There was a poor response to various chemotherapeutic regimens. AFP levels became negative during the course of disease paralleling the disappearance of the YST component and the overgrowth of an increasingly anaplastic MC. The patient died 1 year after diagnosis. We think that, in these cases, MC represented an unusual form of endodermal differentiation of the YST. It is important to differentiate the yolk sac and carcinoid components due to their different responses to chemotherapy and to evaluate the possibility of mucinous carcinoid developing into a highly aggressive carcinoma.

Recent Advances in the Pathology and Classification of Ovarian Germ Cell Tumors

“…In some tumors, such an appearance may predominate and mimic a Krukenberg tumor (54). Mucinous carcinoid may rarely occur in association with ovarian yolk sac tumors (55).…”

Yolk sac tumours revisited. A review of their many faces and names

“…71,72 YSTs can differentiate into an extensive epithelial secondary pattern resembling mucinous carcinoid (adenocarcinoid), displaying prominent goblet cells and neuroendocrine components. 73 This should be differentiated from metastases of amphicrine digestive tract neoplasms to the ovary.…”

Germ Cell Tumors of the Ovary: An Update

“…Rarely, the mature intestinal component of YS(PE)T may give rise to a mucinous carcinoid. 50 Yolk sac tumors can originate from malignant stem cells present in somatic tumors of the ovary and uterus, usually endometrioid adenocarcinoma 51 ( Figure 5, A) and carcinosarcoma. 15 The histology of these unusual YS(PE)Ts is identical to that of tumors of germ cell origin.…”