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Pathology & Laboratory Medicine

URMC / Pathology & Laboratory Medicine / Education / Case of the Month / Case 49: Bilateral Ovarian Masses / Diagnosis

Diagnosis and Discussion

Diagnosis

Primary Low-grade Neuroendocrine Tumor (Carcinoid, Trabecular Type) of Left Ovary and Bilateral Mature Cystic Teratomas of the Ovaries.

Discussion

Most well-differentiated neuroendocrine tumors (WDNETs) occur in the gastrointestinal tract, while the second most common site is the lungs [1]. In the ovary, unlike other organ systems, the term carcinoid is retained for WDNETs. Ovarian carcinoid tumors are rare, accounting for only 0.3 to 1 percent of all WDNETs, and 0.1% of all ovarian neoplasms [2]. They may occur in pure form (15%), where they are considered to be a monodermal teratoma in the ovary, or more commonly in association with other teratomatous components (85%) such as a dermoid cyst [3]. The pure carcinoids are divided further into insular, trabecular, and mucinous types, and resemble WDNETs of the gastrointestinal tract. The most common type is the insular type [4]. The typical symptoms of carcinoid syndrome (facial flushing, diarrhea, bronchospam, hypertension and edema) have been described more commonly in the insular type and less often in the trabecular type [5] .

WDNETs in the ovary, may be primary or metastatic [1]. Of the primary tumors, only 16% have contralateral cystic teratoma of the ovary [6]. Multiple sections are often required to identify the neuroendocrine tumor component when there is a mature cystic teratoma [7].

The optimal treatment strategy remains challenging especially when preservation of fertility is important [7]. According to the literature, the prognosis of organ-confined tumors is excellent for primary ovarian carcinoid tumors and the 10-year survival rates are approximately 100 %. However, if advanced stage, the 5-year survival rate decreases to 33 % [8].     

In conclusion, a primary ovarian carcinoid tumor arising in mature cystic teratoma is very rare in young patients. A perioperative diagnosis is often insufficient to call it primary and hence, mutiple sections are often required to ensure the primary nature of the tumor.

References

  1. Boyraz G, Selcuk I, Guner G, Usubutun A, Gunalp GS. A primary insular type carcinoid arising in a mature cystic teratoma of the ovary: a case report. J Clin Case Rep. 2012; 2:16

  2. Ting Wh Fau- Hsiao, S.M., et al., Primary carcinoid tumor of the ovary arising in a mature  cystic teratoma: a case report. 

  3. Robboy SJ, Scully RE. Strumal carcinoid of the ovary: an analysis of 50 cases of a distinctive tumor composed of thyroid tissue and carcinoid. Cancer 1987;60: 178-182.

  4. Taleraman A, Kurman RJ. Blaustein’s pathology of the female genital tract. 5. New York: springer; 2010.

  5. Davis KP, Hartmann LK, Keeney GL, Shapiro H. Prmiary ovarian carcinoid tumors. Gynecol oncol 1996;61:259-265.

  6. Robby SJ, Norris HJ, Scully RE. Insular carcinoid in the ovary. A clinicopathologic analysis of 48 cases. Cancer. 1975; 36:404-18

  7. Kim JY. A carcinoid tumor arising from a mature cystic teratoma in a 25-year-old patient: a case study. World J Surg Oncol. 2016 Apr 21; 14:120.

  8. Kopf B, Rosti G, Lanzanova G, Marangolo M. Locally advanced ovarian carcinoid. J Exp clin Res.2005; 24:313-6

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