Primary Mucinous Carcinoid Tumor of Ovary

A Case Report and review of literature

ABSTRACT

Primary mucinous (goblet cell) carcinoid tumor of ovary is a rare tumor; only a small

number of cases has been reported in the literature so far. Detailed knowledge of this

tumor is still incomplete. Microscopically, it is similar to mucinous carcinoid tumor

arising in the gastrointestinal tract or elsewhere and, therefore, ruling out the presence of

a primary elsewhere is always of paramount importance. Characteristically the primary

ovarian tumor is associated with other teratomatous components in the majority of the

cases. We report a case of primary ovarian mucinous carcinoid tumor in a 22 year old

female associated with foci of signet ring cell carcinoma and discuss its features in the

context of the current literature.

REPORT OF A CASE

A 22-year-old female Gravida1, Para1 presented to her physician with right lower

quadrant pain for approximately two weeks. On physical examination, a large

mobile mass in the lower abdomen was palpated, arising in the pelvis and

extending 9 cm above the umbilicus. Vital signs and the examination of the other body

systems was unremarkable. The patient denied any bowel or bladder problems. Her

social history included smoking of 4-5 cigarettes per day but she denied the use of

alcohol or any illicit drugs. Her past medical history consisted of tonsillectomy,

appendectomy and cholecystectomy. Family history was significant only for breast cancer in

her maternal grandmother. A CT-scan of the abdomen revealed a large pelvic mass,

measuring greater than 24 centimeters in diameter. It was a complex mass with both solid

and cystic areas. No evidence of adenopathy or ascites was found. The patient underwent

exploratory lapartomy, left salpingo-oophrectomy and right ovarian cystectomy.

MATERIAL AND METHODS

CYTOLOGICAL EXAMINATION

The peritoneal fluid material was smeared on several slides and fixed in 95% alcohol and

stained with papaniocolaou stain. The needles were rinsed in tube of Preserve-Cyte

solution and the tube was held for cell block preparation.

HISTOLOGICAL EXAMINATION

Paraffin-embedded sections of formalin-fixed tissue were studied by routine histology

using hematoxylin-eosin stain.

PATHOLOGIC FINDINGS

GROSS

The specimen was an ovarian mass measuring 28x19x10 centimeters, On

sectioning it consisted of multiloculated cysts ranging in size from 0.5- 6.5 cm in

diameter (Fig. 1). These cysts were filled with mucoid material . One of the cysts, on

sectioning, contained sebaceous material along with hairs consistent with a benign cystic

teratoma. The specimen was generously sampled for microscopic evaluation.

MICROSCOPIC

The microscopic examination of the sections revealed a variety of histologic patterns. The most prominent findings was extraordinary proliferation of glandular structures and abundant mucin production. Some of these glandular structures had the appearance of ordinary benign mucinous cystadenoma (Fig. 2); elsewhere, epithelial cells appeared as aggregates or nests; mucin producing goblet cells were present and some cells bore features suggestive of neuroendocrine origin (Fig. 3 & 4). This comprised the mucinous carcinoid tumor. Multiple foci of clustered signet ring cells were also seen, suspended in mucin lakes (Fig. 5).

A small focal benign cystic teratoma was also identified (Fig. 6), as expected from the

gross inspection. This constellation of findings, from mature benign cystic teratoma, to

goblet cell carcinoid, to clusters of signet ring cells, was confirmed to be the components

of the single neoplastic ovarian entity of primary mucinous carcinoid tumor.

COMMENTS

Primary carcinoid tumors of ovary are rare tumors comprising less than one percent of

primary ovarian neoplasms. These tumors have been divided into four types on the basis of

morphologic and clinicopathologic features: insular, trabecular, strumal, and mucinous

(goblet cell) [1,2,9]. These tumors are microscopically similar to carcinoid tumors of

gastrointestinal tract or elsewhere; however, in the ovary, they are associated with other

teratomatous components in 85-90 percent of the cases. In the remainder, the carcinoid

component is the exclusive element of the tumor and should be carefully distinguished from

metastases from some other primary site [1,2,9]. The mucinous (goblet cell) type of

primary ovarian carcinoid tumor is much less reported in the literature than the other three

types of primary carcinoid tumors of ovary [1,2,9]. Only a few cases were reported in

detail in the literature until recently, when Baker PM, Olivia E, Young RH, et al in 2001[2]

published an extraordinary report of seventeen cases. These neoplasms are primarily

composed of small glandular structures, acini or nests containing a prominent population of

two types of cells: goblet cells and; neuroendocrine cells. The age distribution is similar to

neuroendocrine tumors of other organs, but, unlike some others, the mucinous variety has

not found to be associated with the carcinoid syndrome [2, 9]. Grossly, the tumor

resembles other ovarian carcinoids and is almost always associated with a benign mature

teratoma. However, unlike other such primary tumors, this variety is predominantly cystic.

The typical microscopic appearance is that of glands with small or inconspicuous lumina or

nests, floating in pools of mucin. The stroma may be scanty or abundant and

fibromatous.[1,2,9,12] Hybrid cells containing both mucin and neuroendocrine granules may

be present. The nuclei are uniform, small, and round to oval. Small pools of mucus may lie

within cystically dilated glands or within stroma. Baker, et al [2] divided these tumors into

well differentiated, atypical or poorly differentiated patterns on the basis of their

microscopic features. As in the present case, the poorly differentiated ones often contain

signet ring cells which may invade the stroma singly or in small nests mimicking the pattern

of a Krukenberg tumor.

Mucinous carcinoid tumors were first described as tumors intermediate between carcinoid and an adenocarcinoma in the appendix by Gange, et al [1,4] who reported three cases in 1969. Then Klein and Rosen [6] and Subbuswamy, et al [10] described another series of these tumors under the terms "mucinous carcinoids" and "goblet cell carcinoids" respectively. Since then a number of detailed cases of appendiceal mucinous carcinoid tumor, metastatic to ovary, have been reported [3,5,6,7,8].
Therefore, distinguishing a primary mucinous carcinoid from a secondary (metastatic) one, from appendix or elsewhere, should always be considered important. This distinction is made on the basis of surgical and pathologic criteria. Similarly, the same criteria facilitate the distinction of a primary mucinous carcinoid with a signet ring carcinomatous component from a Krukenberg tumor. In the absence of teratomatous elements, a primary tumor can be very difficult to distinguish from metastases. Some of the observations favoring, or establishing, the diagonosis of a metastatic origin in such cases, include: the probable or certain presence of an intestinal carcinoid tumor; bilateral involvement; intra-ovarian growth as multiple nodules; extraovarian metastases, particularly to mesenteric lymph nodes and liver, and; laboratory, or clinical, evidence of carcinoid syndrome most commonly expressed by increased levels of circulating and urinary 5-HIAA. The synchronous presence of other teratomatous components much favors a primary origin of the tumor. These tumors are treated as an ovarian neoplasms with malignant potential and, hence, bilateral salpingo-oophrectomy is commonly employed. However, Baker PM, Olivia E, Young RH, et al [2,12] discussed, in their report, that women with stage I, who want to preserve their ovarian function, can elect a unilateral salpingo-oophrectomy with close followup. The appendix should be routinely removed during the surgery for thorough pathologic evaluation. Staging should be performed if the diagnosis is suspected or confirmed by intraoperative frozen section examination.

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