Ovarian undifferentiated carcinoma with voluminous mesenteric presentation

Abstract

INTRODUCTION

About 5% of ovarian cancers are so poorly differentiated and difficult to classify that they are called undifferentiated carcinomas and usually have disseminated disease at presentation. Extra pelvic debulking it is difficult to complete.

PRESENTATION OF CASE

We report a case of a rare ovarian tumor presented as a large mesenteric tumor of 14 cm diameter in a 73 years old woman.

DISCUSSION

Undifferentiated carcinomas are usually large, solid with hemorrhage and necrosis, bilateral and most are difficult to classify histologically. Rarely are pure, generally identified through the extensive sampling of lesions, some other components of surface epithelial carcinoma and usually the predominant element is the latter. Cases with predominantly undifferentiated component are rare.

CONCLUSION

The treatment and diagnostic approach is the same as for other high-grade epithelial tumors of the ovary, but in this particular case the differential diagnosis and diagnostic approach is that of a mesenteric tumor.

1. Introduction

About 5% of ovarian carcinomas are so poorly differentiated and difficult to classify that are designated “undifferentiated”.^1,2 In most cases this type of tumor has spread widely beyond the ovary at diagnosis, made it difficult to complete resection of the tumor and its implants or metastases. Undifferentiated carcinoma possess the worst prognosis of tumors of ovarian surface, are very rare with most cases reported as single case, however, in a large series 29 of 35 patients died of tumor within 3 years, and eventually five of six survivors died.³ We report a case of undifferentiated carcinoma arising in a small component of papillary serous ovarian carcinoma that manifested as a large mesenteric tumor.

2. Case report

A Mexican woman 75 years old with a history of hysterectomy for leiomyomas 50 years ago, who started a week before admission with acute abdominal pain and decreased stool frequency, so she was taken to an exploratory laparotomy in a community hospital. The intraoperative finding was a 14 cm diameter tumor in the mesentery that was subsequently diagnosed in biopsy as poorly differentiated malignant neoplasm. She was referred to our hospital where physical examination and abdominal computed tomography showed a mesenteric solid-cystic neoplasm with necrosis apparently arising from intestinal wall, no injuries reported in other organs. The metabolic status was normal; the tumoral markers CA-125, CA19-9 and Carcinoembrionic Antigen (CEA) were in normal parameters and Karnofsky performance status scale rating 90% (able to carry on normal activity; minor signs or symptoms of disease). The patient was subject to a new laparotomy with intraoperative findings of ovarian cysts and pelvic wall adhesions, implants in cecal appendix, cecum, and a 14 cm tumor in mesentery wrapped by small and large intestine with adhesions. Resection of the lesion, bilateral salpingo-oophorectomy, right hemicolectomy and debulking was performed. Macroscopically there was a mesenteric tumor with dense adhesions to the bowel wall (Fig. 1A), the surface appearance is a tumor with solid and cystic hemorrhagic areas (Fig. 1B), which shows extensive areas of necrosis. In the serosa of the cecum and mesenteric lymph nodes observed similar histologic characteristics. The right ovary was completely replaced by a unilocular cystic neoplasm with serous content and a thin-wall with a solid area of 13 mm × 12 mm with white papillary formations. Microscopically in the tumor and the implants was observed a malignant small round cell neoplasm with solid pattern, wide necrosis and atypical mitoses, this neoplasm was focally positive for EMA and negative for cytokeratins AE1/AE3, CK7, CK20, vimentin, desmin, actin, CD117, CD34, p53, S-100, CA-125 and calretinin, findings that are compatible with undifferentiated carcinoma. In the ovary sections from the solid areas were identified malignant papillary and micropapillary formations invading the ovarian stroma (Fig. 2C). In the stroma of papillae is an undifferentiated spindle cell neoplasm with the same characteristics as that observed in the mesentery. Additionally, we performed electron microscopy study of the mesenteric tumor, which demonstrated an undifferentiated cell that has little primitive intercellular junctions (Fig. 3) confirming the diagnosis of undifferentiated carcinoma. The final pathology report was undifferentiated carcinoma with focal component (<5%) of high grade ovarian serous papillary carcinoma with involvement of the right ovary, vermiform appendix, cecum, mesentery and metastasis in 15 of 33 lymph nodes (mesenteric and pericolonic).

Fig. 1.

Surgical specimen showing the tumor wrapped mesenteric bowel loops (A). The neoplasm has extensive areas of necrosis (B).

Fig. 2.

Photomicrographs of malignant neoplasm of small round cells with focal spindle areas (A). With focal and weak expression of EMA (epithelial membrane antigen) (B). Ovarian high grade papillary serous carcinoma (lower right) with type 2 micropapillary component (top left) (C).

Fig. 3.

Undifferentiated neoplasm of polygonal cells with condensed chromatin central, few organelles, poorly defined cytoplasmic membranes. At the center of the image are few desmosome junctions.

The patient had a good postoperatory and is currently alive and without evidence of tumor activity at 12 months after surgery.

3. Discussion

Mesenteric tumors are rare. Most are metastatic, but can be found a wide variety of types from connective tissue, epithelial, mesothelial and germ cells. These tumors can be found in any age group, may be solid or cystic. Among the solid tumors can be found mesenteric lipomas, malignant tumors (leiomyosarcoma and liposarcoma), desmoid tumors, primary lymphoma, carcinoid tumors and others. Among the cystic tumors include urogenital, enteric, traumatic, gas, fungal, parasitic and tuberculosal cyst, in children can be congenital.^4,5 The symptoms include increased abdominal girth, vague abdominal pain and postprandial fullness. These symptoms lead to making imaging studies where such tumors are identified, with computed tomography as excellent diagnostic tool with high sensitivity and accurate information regarding the size, location, structure and length of the tumor. Most primary mesenteric tumors are of mesenchymal origin⁶ and histologically benign and should be considered the next: (1) desmoid tumor or mesenteric fibromatosis. They are rare tumors, non-encapsulated, do not metastasize but are locally aggressive with capacity to infiltrate or recurr. The most frequent location is the mesentery of the small intestine. They appear commonly in women of childbearing age, particularly frequent in patients with familial adenomatous polyposis (Gardner syndrome). (2) Mesothelioma. It is rare, usually fatal, arises from the serous lining of the peritoneum. Most patients have a history of asbestos exposure. (3) Primary mesenteric sarcomas: are very rare tumors, usually in old adults. The secondary lesions could be lymphoma, neuroendocrine neoplasms, metastasis of carcinomas and gastrointestinal stromal tumor (GIST), which often is connected to the wall of a loop of intestine and metastasis of epithelial tumors usually present as peritoneal carcinomatosis and exceptionally as bulky implants. If an implant is very large one most suspect ovarian origin.

Also, the differential diagnosis should be established with non-neoplastic processes such as sclerosing Mesenteritis and trauma or previous surgery changes.

The macroscopic differential diagnosis of the specimens is difficult and raises several differential diagnoses, the main of them is a GIST because is a mesenteric neoplasm with apparent relationship to the intestinal wall and when the GISTs are greater of 10 cm they are prone to cystic degeneration, hemorrhage, metastatic implants and lymph node metastasis.⁷ The right ovary is small and almost cystic except for a small mural nodule, so that the initial diagnosis to consider is a serous tumor, mainly borderline. Generally implants of serous carcinomas are microscopic and rarely are voluminous.⁸ The most likely diagnosis is that of a primary mesenteric sarcoma, since it is generally characterized by their large size and may have hemorrhage, necrosis and metastases, and in this context, the ovarian tumor could be considered as a synchronous serous tumor.

Undifferentiated carcinomas of the ovary not exhibit characteristics that allow classified into any of the types of carcinoma of ovarian surface epithelium.⁹ Pure cases are rare and up to 80% of tumors classified as undifferentiated carcinomas may contain small foci of a recognizable subtype of carcinoma, usually serous or endometrioid adenocarcinoma. When strict pathological criteria are used, this diagnosis is very rare, comprising <1% of ovarian carcinomas. This neoplasm is so little differentiated that immunohistochemical reactions are negative for almost any marker and only focally positive for epithelial markers as in this case are found in a very small proportion.¹⁰ The clinical presentation is similar to that of other carcinomas and in 15% of cases are bilateral. Ninety-one percent of cases are detected in advanced stages. Rarely, undifferentiated carcinomas are small cell or neuroendocrine features. The histologic differential diagnosis are granulosa cell tumor, transitional cell carcinomas, poorly differentiated squamous cell carcinoma, small cell carcinomas, lymphomas and metastatic carcinomas. The survival rate at 5 years varies between 17% (stage III) and 68% (stage I), with an overall average of 6%. The prognosis for these patients is very dark so it should be surgically staged and debulking these patients to improve their survival.¹¹ The diagnosis of metastatic ovarian carcinoma should be considered in the differential diagnosis of mesenteric tumors.

Conflict of interest statement

None.

Funding

None.

Ethical approval

Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Author contributions

Rosa Angélica Salcedo-Hernández and David Cantú de León – write the paper. Clinical discussion. Leonardo Saúl Lino-Silva and Maria Delia Pérez-Montiel – write the paper Histopathological discussion. Kuauhyama Luna-Ortiz – write and reviewed the paper for correct spelling and grammar, Apports to clinical discussion.