Primary carcinoid tumor of the ovary: report of an unusual case

Carcinoid tumors are endocrine malignancies that are often associated with a characteristic syndrome, the malignant carcinoid syndrome, which is most common in patients with small bowel tumors and liver metastases. In the rare instances when the syndrome is present without liver metastases the primary tumor is usually localized to the bronchus or ovary and secretes hormones directly into the systemic circulation. About two thirds of patients with carcinoid syndrome have evidence of carcinoid heart disease. We report on a case of a primary ovarian carcinoid tumor with an unusual clinical presentation.     

Insular carcinoid of ovary associated with malignant mucinous tumors

Ovarian carcinoids are frequently encountered in association with mucinous cysts and occasionally with mucinous cystadenomas. From a series of more than 200 ovarian carcinoids, this report describes 2 cases of insular carcinoid, 1 of which arose in a cystadenoma of borderline malignancy and the other in a mucinous adenocarcinoma. Histogenesis, histologic patterns, and natural history of carcinoid are discussed.     

Primary hepatic carcinoid tumor

We report the case of primary hepatic carcinoid tumor of which diagnosis was made by fine needle biopsy of a liver mass. The patient was treated successfully by left hepatic trisegmentectomy. This patient presented with complaints of generalized fatigue, but denied the presence of flushing, diarrhea, or other endocrine symptoms. Physical examination was unremarkable. A biopsy specimen revealed Grimelius stained cells that were immunoreactive for chromogranin A. Careful pre- and intraoperative examinations revealed no other primary lesions. Argyrophilia of the tumor cells suggested that the tumor was of fore five cases of primary hepatic carcinoid tumors previously reported in the literature are also reviewed.     

卵巢原发甲状腺肿类癌的临床病理分析

目的:探讨卵巢原发甲状腺肿类癌的临床病理特征.方法:观察和分析2例卵巢原发甲状腺肿类癌病例的病理组织学及免疫表型的特点,并复习相关文献进行讨论.结果:例1卵巢类癌伴有成熟性囊性畸胎瘤成分,例2为单纯甲状腺肿类癌,类癌成分呈岛状与小梁状生长,甲状腺滤泡成分与类癌相互混杂.类癌的细胞比较一致,胞浆嗜酸性,染色质均匀,核分裂象少见.免疫组化2例类癌成分CK(+),CK19(+),CD56(+),NSE(+),Syn(+),TTF-1和TG散在阳性,甲状腺成分TTF-1(+)、TG(+).卵巢甲状腺肿类癌应与甲状腺癌鉴别,甲状腺癌的肿瘤细胞有高分裂活性,没有神经内分泌分化,细胞岛状或梁状分布不明显.结论:卵巢甲状腺肿类癌是罕见的低度恶性肿瘤,预后较好,病理形态学观察和免疫组化染色有助于明确诊断.     

Krukenberg tumor of ovary with features of mucinous cystadenocarcinoma

An unusual case of bilateral Krukenberg tumors having a predominant multicystic mucinous component which on gross and microscopic examination resembled a mucinous cystadenocarcinoma is presented. It is important to distinguish between these two tumors as Krukenberg tumours have a significantly worse prognosis.     

Strumal carcinoid of the ovary.

A 45-year-old woman presented with an apparently nonfunctioning strumal carcinoid of the ovary. On ultrastructural examination the cells in the solid parts of the tumor as well as in the "follicular" areas contained dense-core secretory granules, indicating that this neoplasm is a pure carcinoid tumor. The thyroid-like appearance in areas is due to the accumulation of secretory material within unusually dilated acinar spaces. This study raises the question whether any strumal carcinoid contains thyroid tissue since the cells forming the follicle-like spaces in this tumor contain dense-core granules.     

Primary carcinoid tumor of the uterine cervix associated with positive serotonin reaction

A 46-year-old female with primary cervical carcinoid is reported. She was well with no recurrence for more than 5 years after surgery and radiation therapy. Pathologically, the tumor was composed of solid nests including rosette-like structures and tubular formations. Multiple argyrophil granules and a few argentaffin granules were revealed histochemically and serotonin was demonstrated by formalin-induced fluorescence.     

Primary mucinous carcinoma

Primary mucinous carcinoma of the skin is a rare entity. It usually presents as a painless nodule, cyst, or ulcer in the head and neck region (especially the eyelid). These tumors have a low metastatic potential but tend to recur locally. Histologically, groups of light- and dark-staining epithelial cells form cords, nests, and ducts separated by clear areas representing sialomucin. Due to tumor continuity and a low rate of lymphatic or hematologic spread, Mohs micrographic surgery appears to be an effective treatment. Herein, two cases of primary mucinous carcinoma are presented.     

Treatment of the carcinoid tumor and the malignant carcinoid syndrome

Certainly in treatment of the carcinoid tumor surgery has a well-established curative and palliative potential. The primary challenge is a knowledgable marriage between stage of disease and aggressiveness of operative procedure. Nonsurgical treatment of the malignant disease per se has thus far not produced optimum results and, in the opinion of this author, should still be confined to a clinical research setting. It would seem very doubtful, however, that the more mundane types of trials, empirically testing drug after drug and arbitrarily concocted drug combinations, is the most productive road to follow. There is an evident need for more sophisticated approaches to this tumor, which has unique metabolic characteristics that should be pharmacologically exploitable. Past experience has indicated that such a setting may permit dramatic therapeutic accomplishment. There is a strong need for animal models or established cell lines which would facilitate preclinical therapeutic exploration. The carcinoid syndrome presents an unparalleled opportunity for fundamental physiologic observations and for experimental therapeutic study that can have applicability not only in palliating the syndrome itself but also in the management of other pathophysiologic states that may involve more subtle abnormalities of the same hormonal mechanisms. The patient with the carcinoid tumor should not just be a fascinating curio for grand rounds exhibition. He should be a focal point for research involving an experienced, multidisciplinary clinical team supported by devoted basic scientists. If our patient resources and efforts can be concentrated in this manner, the carcinoid should be a strong candidate for the next medically curable human cancer.     

Primary chemotherapy in strongly suspected yolk sac tumor of the ovary

We report a case of a right ovarian tumor measuring 10.8 × 10 × 8.5 cm (diagnosed as a yolk sac tumor) in a 31-year-old nulligravid woman with serum α-fetoprotein (AFP) level of 25 000 ng/ml and pleural effusion. Seven courses of primary chemotherapy (PVB; cisplatin, vinblastine, bleomycin) were given from May 16 to November 11, 1994. No surgical procedures were conducted. After the second course of PVB, the pleural effusion resolved; after the third course, the tumor disappeared, and after the fourth course, serum AFP decreased to the normal range. At the true of writing (May 11, 1998), the patient is in good health without recurrence, with an AFP level below 20 ng/ml. This paper reports, for the first time, a patient with yolk sac tumor in whom remission was achieved by primary chemotherapy alone. Received: September 3, 1997 / Accepted: June 3, 1998     

Endodermal sinus tumor and mucinous cystadenofibroma of the ovary. Occurrence in an 82-year-old woman

We encountered an unusual ovarian tumor consisting of a mixture of typical endodermal sinus tumor (EST) and mucinous cystadenofibroma that occurred in the ovary of an 82-year-old female patient. The EST component showed the classic histologic features of this tumor. Serum alpha-fetoprotein (AFP) level was not determined. Tumor stains were negative for AFP but positive for alpha-1-antitrypsin. The malignant germ cell component was intimately associated with the benign mucinous component. Focal production of epithelial mucin and carcinoembryonic antigen (CEA) in the EST component suggested a probable association between the two tumor types. The tumor was confined to one ovary, and the patient is disease-free 2 years after surgical therapy. This neoplasm is unique not only for the malignant germ cell component occurring in an 82-year-old woman, but for the unusual combination of tumor types. The pathogenesis is unknown.     

Primary carcinoid tumour of the pancreas

Pancreatic carcinoid tumours are extremely infrequent. Usually, the biological behaviour is indolent and diagnosis is late and often casual. We present the case of a patient initially diagnosed as having liver metastasis of unknown origin. PET identified a primary pancreatic site and the initial histologic diagnosis was adenocarcinoma. Following an uncertain response to chemo- and radio-therapy the repeat histologic assessment indicated a carcinoid tumour of the pancreas. After complete surgical resection and liver transplantation, patient remains free of disease. Conclusions: The co-existence of several diseases with similar morpho-structural features makes diagnosis complicated. PET is of uncertain use in the evaluation of carcinoid tumours, and is considered inferior to¹¹¹Indium-octreotide scan. The only curative treatment is surgical resection, with liver transplantation as a valid option in the treatment of these tumours.     

Invasive carcinoid tumor of the heart

Carcinoid tumors have been described in almost every organ and may affect virtually every body system. Cardiac involvement manifesting as right-sided valvular disease is characteristic of the carcinoid syndrome; however, direct myocardial involvement is unusual. We present a case of an invasive carcinoid tumor whose primary manifestation was myocardial invasion. J. Surg. Oncol. 1997;66:264–266. © 1997 Wiley-Liss, Inc.     

Primary retroperitoneal mucinous cystadenocarcinoma

A case of primary retroperitoneal cystadenocarcinoma is presented as the fourth reported case in the world literature to date. The cyst was removed intact and demonstrated an infiltrating malignant process with nuclear pleomorphism and mitotic activity. No ovarian tissue was identified and a cancer antigen 125 (CA 125) test was normal. The patient underwent a staging procedure subsequently that included peritoneal washings, hysterectomy, bilateral salpingoophorectomy, and iliac node dissection. No metastases were found and the patient is without recurrence 22 months postoperative. The literature is reviewed to better define the origin and prognosis of these tumors.     

Reduced vascular basement-membrane immunostaining in mucinous tumours of the ovary

Tumour vasculature is heterogeneous, exhibiting a range of vessel densities and the vascular basement membrane (VBM) of tumour blood vessels may be fragmented or absent. Increased microvessel density (MVD) has been reported in mucinous ovarian tumours as compared with other histologic sub-types. We hypothesized that VBM immunostaining differs between regions of the ovarian tumour vasculature and between ovarian tumour types exhibiting different MVD. Serial sections from 56 ovarian tumours were immunostained using antibodies to the VBM components collagen IV, heparan sulphate proteoglycan and laminin, and the endothelial cell marker CD31. Regions of high and average MVD were selected, and the number of vessels positive for each VBM component were counted and expressed as a percentage of the number of CD31-positive vessels. The percentage of VBM-positive vessels did not differ between the high and average MVD regions of borderline or malignant, mucinous and serous tumours. The percentage of VBM-positive vessels in mucinous tumours was less than that observed in malignant and borderline serous tumours and benign tumours (p < 0.02). Possible explanations for these findings are (i) that VBM turnover is similar throughout the vasculature; (ii) that the VBM is present both during angiogenesis and in the newly formed vessels of high MVD regions; or (iii) that an alternative angiogenesis mechanism is utilized in different ovarian tumour types, or even between different regions of the same tumour. Int. J. Cancer (Pred. Oncol.) 79:139–143, 1998.© 1998 Wiley-Liss, Inc.