Only rare primary mucinous (goblet cell) carcinoids of the ovary have been reported, and their clinicopathologic features have not been well delineated. The authors studied 17 examples from patients 14 to 74 years of age. The clinical presentations were similar to those of ovarian neoplasms in general. The tumors ranged from 0.8 to 30 cm in diameter. In six cases the tumor was in the wall of a mature cystic teratoma, appearing grossly as solid nodules or areas of thickening in four of them, six tumors were entirely solid, and five were solid associated with other types of cystic tumor. The tumors were divided into three groups on the basis of their microscopic features. Six neoplasms, designated "well differentiated," were composed of small glands, many of which floated in pools of mucin. The glands were lined by goblet cells and columnar cells, some of which were of neuroendocrine type. Three tumors, designated "atypical," were characterized by crowded glands, some of which were confluent, small islands with a cribriform pattern, and scattered microcystic glands. The glands were lined by cuboidal to columnar cells, some of them neuroendocrine, admixed with goblet cells. Eight tumors, designated "carcinoma arising in mucinous carcinoid," contained islands and larger nodules of tumor cells, or closely packed glands, as well as single cells, mainly of the signet ring cell type. Most of the cells were devoid of mucin and were severely atypical with marked mitotic activity. Necrosis was present in all eight tumors. Seven of the eight tumors with a carcinomatous component contained at least minor foci of well-differentiated mucinous carcinoid; the eighth contained only foci of atypical mucinous carcinoid. The neuroendocrine nature of a variable proportion of the cells in all three groups was demonstrated by staining for neuroendocrine markers. The mucinous nature of other cells was confirmed by mucicarmine or Alcian blue stains. The ovary contained an intrinsic component of trabecular and insular carcinoid, and of strumal carcinoid in one case each, an adjacent mature cystic teratoma in six cases, mucinous cystadenocarcinoma in three cases, and borderline mucinous cystic tumor, borderline Brenner tumor, and epidermoid cyst in one case each. Fifteen tumors were stage I, one was stage II, and one was stage III. The last two tumors had a carcinomatous component. Follow-up data were available for 15 patients; 12 were alive and free of tumor 2.3 to 14 years (average, 4.7 years) after the ovarian tumor was excised. One patient, whose tumor had a carcinomatous component, died 3 years postoperatively of unrelated causes. Two patients, both of whom had a carcinomatous component in their tumor, died 9 and 12 months postoperatively. Primary mucinous carcinoids must be distinguished from metastatic mucinous carcinoid tumors from the appendix or elsewhere. Features supporting an ovarian origin are the additional presence in the specimen of teratoma or an ovarian surface epithelial tumor, an absence of blood vessel or lymphatic space invasion, and confinement to a single ovary. Similar features help to distinguish mucinous carcinoids from Krukenberg tumors. Mucinous carcinoids should also be distinguished from strumal carcinoids, which can contain mucinous glands, and insular carcinoid tumors that arise rarely in the wall of a mucinous cystic neoplasm. Although the number of cases in this series is small, the follow-up data suggest that the degree of differentiation, particularly the presence of frank carcinoma, is an important prognostic factor.
Pathologic staging determines the management of patients with endometrial adenocarcinoma and uterine sarcomas following the initial surgery and it is an essential component of the initial assessment. FIGO stage, tumor subtype, grade of differentiation, myometrial invasion, lymphovascular invasion, and other factors that guide the treatment decisions covered in the subsequent chapters are extensively discussed.
This essay considers selected peritoneal lesions many of which were the subject of studies coauthored by Dr Robert E. Scully. His article on multilocular peritoneal inclusion cysts has largely led to these lesions being considered non-neoplastic, eschewing the term cystic mesothelioma. These cysts are often associated with reactive mural mesothelial proliferations that can potentially lead to a misdiagnosis of mesothelioma. Clinical findings, such as a common association with endometriosis or prior operations, can prompt consideration of a reactive lesion. Mesothelial hyperplasia may be difficult to distinguish, when florid, from mesothelioma but a variety of gross and microscopic features will aid their recognition. Nodular peritoneal aggregates of histiocytes (sometimes admixed with mesothelial cells) may occasionally be a striking finding that can be misdiagnosed as a metastasis if the patient has a known neoplasm. Appreciation of their bland nuclear features and histiocytic nature, confirmed by immunohistochemical markers, facilitate the diagnosis. Various forms of peritonitis are briefly considered including sclerosing peritonitis, a process sometimes associated with luteinized thecomas (thecomatosis) of the ovaries, an entity first appreciated by Dr Scully. Mesotheliomas are briefly reviewed emphasizing the caution that should be used in applying the designation "well-differentiated papillary mesothelioma." Many interpret the latter as benign, but multifocal lesions must be thoroughly examined histologically because of potential overlapping features with malignant mesothelioma. The morphologic spectrum of malignant mesothelioma and its usually straightforward distinction from müllerian neoplasms is considered, as is its occasional presentation as a dominant ovarian mass. The spectrum of low-grade serous peritoneal neoplasms including the "psammocarcinoma" is reviewed. Finally, various benign müllerian lesions, particularly endometriosis and endosalpingiosis, may be conspicuous in peritoneal specimens and sometimes are grossly striking. The usual presence of benign endometrioid epithelium and stroma should facilitate the correct diagnosis of endometriosis, but in cases in which the stroma is atrophic or the sole component (stromal endometriosis), diagnostic problems may arise.
Prominent benign vascular proliferations associated with neural tissue in five cases of ovarian teratoma are described. The ages of the patients ranged from 15 to 35 years. Three of the five had tumors confined to the ovary, one had peritoneal implants, and one had widespread metastatic immature teratoma. Two of the patients are alive and well, 8 and 9 years postoperatively. Follow-up is unavailable in two cases and the final case was recent. The tumor in three of the cases had features of mature cystic teratoma including abundant mature neural tissue and, in one instance, microscopic foci of primitive neuroepithelium. The tumor in the fourth case was an immature teratoma with abundant primitive neuroepithelium, and in the fifth case was a mixed germ cell tumor, composed mostly of immature teratoma with a minor component of yolk sac tumor. In all the tumors there was a prominent vascular proliferation composed of long thin-walled, curved vessels or a solid glomeruloid arrangement. Immunohistochemistry done in two cases confirmed the vascular nature of the proliferation. Angiogenesis, likely as an expression of vascular endothelial growth factors, is a well-known phenomenon in a variety of neural and neuroendocrine neoplasms, in particular high-grade gliomas. However, very few cases of this phenomenon have been described in association with neural tissue in the ovary. Recognition of this proliferation as a benign secondary one is important to avoid misdiagnosis of a vascular neoplasm or an immature teratoma, as happened in one of our cases.
Seventy-five malignant mesotheliomas of the peritoneum in women were reviewed to highlight their morphologic spectrum. The patients ranged from 17 to 92 (mean, 47.4) years of age. The clinical presentation was usually abdominal or pelvic pain, abdominal swelling (sometimes due to ascites), or a pelvic mass. On microscopic examination, the majority of the tumors had only an epithelial morphology, but 4 were biphasic and 1 was sarcomatoid. The most common epithelial patterns were tubular and papillary (which often coexisted), but 5 tumors were purely diffuse; 2 had cells with abundant glassy eosinophilic cytoplasm (so-called deciduoid mesothelioma). The cells in the tubular and papillary patterns were generally cuboidal with scant to moderate amounts of eosinophilic cytoplasm. Nuclear atypia was usually only mild, although a minority of cases had moderate or even, occasionally, severe atypia. Many tumors had foci that, viewed in isolation, resembled so-called well-differentiated papillary mesothelioma, and accordingly that diagnosis should be made cautiously. Unusual features were lymphoid follicles (13 cases), striking myxoid stroma (5 cases), prominent foamy histiocytes (5 cases), and a striking vascular proliferation (1 case). The varied morphology of peritoneal malignant mesotheliomas may raise a broad differential diagnosis, but in most cases the resemblance to other tumors is limited. Histochemistry, immunohistochemistry, and electron microscopy may provide important aid, particularly when tissue is limited, but should be needed only occasionally.
The use of frozen section in gynecological pathology has not been emphasized in the literature to the same degree as in other surgical fields. This review focuses on the indications, contraindications, and limitations of frozen-section diagnosis specific to the female genital tract. An intraoperative consultation in gynecological pathology is indicated (a) to ensure that the tissue sampled is adequate for diagnosis, (b) to determine the nature of a disease process, (c) to plan for appropriate ancillary studies, (d) to determine tumor spread, and (e) to assess the margins. In the ovary, mucinous tumors in particular may present a challenge and potential for misdiagnosis at the time of frozen section. It is important to determine the nature of the ovarian involvement, as tumor size greater than 10 cm or bilateral involvement strongly suggests a metastatic process. Also, the distinction between ovarian carcinoma and tumors of borderline malignancy may be difficult in a limited sampling. In the germ cell category, an important distinction is that of a dysgerminoma from a large cell lymphoma, due to different treatment regimes. Pregnant and postpartum women present a unique challenge as the effects of high levels of pregnancy-related hormones may result in lesions that closely mimic malignancy. Although intraoperative frozen section should be discouraged as a primary diagnostic procedure for endometrial carcinoma, it can be very helpful to identify those patients who are at risk for extrauterine spread and who may require lymphadenectomy. Analysis of a cone biopsy of the cervix by frozen section may be warranted particularly if the previous biopsy showed equivocal stromal invasion, an uncertain depth of invasion, there are issues related to fertility; however, the process is time consuming and may compromise the permanent sections if the lesion is very small. Frozen-section diagnosis in squamous cell carcinoma and in Paget disease of the vulva is infrequently requested as these entities are multifocal resulting in an inaccurate frozen-section diagnosis. Lastly, intraoperative evaluation of lymph nodes including the role of sentinel lymph nodes is discussed.
