| Abstract|| |
Extrapancreatic solid pseudopapillary neoplasms (SPNs) are rare tumors, which bear morphological, immunohistochemical, and molecular features similar to those of pancreatic counterparts. SPN occurs primarily in adolescent girls and young women. It is considered to be a malignant neoplasm with low-grade biology. Ovarian SPNs are uncommon, have benign morphology, usually limited to the ovary and local surgical excision is curative. We report an unusual case of SPN of right ovary with extraovarian spread and metastases to lymph nodes. To the best of our knowledge, this is the second documented case of extragonadal spread of ovarian SPN.
Keywords: Carcinoma, ovary, pancreas, solid pseudopapillary neoplasm
|How to cite this article:|
Gahlot GP, Mridha AR, Sable M, Sharma MC, Pramanik R, Kumar L. Solid pseudopapillary neoplasm of the ovary with metastases to the omentum and regional lymph nodes. Indian J Pathol Microbiol 2016;59:348-50
|How to cite this URL:|
Gahlot GP, Mridha AR, Sable M, Sharma MC, Pramanik R, Kumar L. Solid pseudopapillary neoplasm of the ovary with metastases to the omentum and regional lymph nodes. Indian J Pathol Microbiol [serial online] 2016 [cited 2019 Jun 17];59:348-50. Available from: http://www.ijpmonline.org/text.asp?2016/59/3/348/188107
| Introduction|| |
Solid pseudopapillary neoplasm (SPN) is an uncommon pancreatic neoplasm accounting for 1–2% of all pancreatic malignancies. However, rarely it can occur in ovary, retroperiotoneum, omentum, mesocolon, liver, duodenum, and stomach. Only seven cases of ovarian SPN have been reported in the English literature [Table 1]. Though most of these tumors have benign behavior, an occasional, aggressive ovarian SPN with cytologic evidence of malignancy and disease-associated death has been described. We report a case of morphologically benign appearing SPN with metastases to omentum, parametrium, and pelvic lymph nodes.
|Table 1: Demographic profile of reported cases of ovarian solid pseudopapillary neoplasm|
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| Case Report|| |
A 25-year-old unmarried female presented with a complaint of lower abdominal pain for 4 months at outside clinic. There was no history of fever, dysuria, menstrual irregularity, tuberculosis, diabetes, or weight loss. Past and family history was unremarkable. Pelvic examination revealed a large nontender mass in the right adnexa. Liver, spleen, and peripheral lymph nodes were nonpalpable. Abdominal ultrasonography showed a well-defined 12.0 cm × 6.3 cm right adnexal mass with solid and cystic components on contrast-enhanced computed tomography (CECT) [Figure 1]a and [Figure 1]b. Left ovary and pancreas were normal [Figure 1]c. Chest X-ray, complete blood count, serum biochemistry, cancer antigen-125 (CA-125), beta-human chorionic gonadotropin, α-fetoprotein, CA19-9, and carcinoembryonic antigen were within normal limits. With a clinical diagnosis of ovarian malignancy, the patient underwent right salpingo-oophorectomy, total omentectomy, macroscopic clearance of the tumor, and pelvic lymph node dissection. Tumor deposits on greater omentum, posterior surface of the uterus, bilateral parametria, and pouch of Douglas were observed. Peritoneal fluid cytology showed malignant cells. The uterus, left ovary, and Fallopian tube More Details were preserved as the patient was unmarried. Subsequently, she was referred to our institution. Microscopic examination revealed a neoplasm composed of pseudopapillae lined by polyhedral cells with round to oval vesicular nuclei, inconspicuous nucleolus, and moderate amount of pale eosinophilic cytoplasm. Central fibrovascular cores were present. No mitosis, pleomorphism, or necrosis were seen [Figure 2]a and [Figure 2]b. Omentum, parametria, and pelvic lymph nodes showed tumor metastases. The tumor cells were immunopositive with CD56 (1:100) (Novocastra, UK), CD10 (1:50), cyclin D1 (1:100) (Spring BioScience UK), and β-catenin (1:200) (BD Biosciences US) [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f but negative for epithelial membrane antigen (1:400), calretinin (1:200), synaptophysin (1:200), chromogranin (1:200), smooth muscle actin (1:400), E-cadherin (1:50) (Thermo Fisher Scientific Inc, US), S100 (1:800), glial fibrillary acidic protein (1:1000) (Dako, US), alpha-inhibin (1:50), and progesterone receptor (PR) (1:300) (Spring BioScience UK). No chemotherapy was given. No recurrence, ascites, or any enlarged lymph nodes were seen on CECT images for the last 18 months.
|Figure 1: Contrast-enhanced computed tomography axial and sagittal images show heterogeneous large right ovarian mass with enhancing solid and nonenhancing cystic components (a and b). The pancreas is normal (c)|
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|Figure 2: H and E stain sections show solid (a, ×100) and pseudopapillary pattern (b, ×200). The tumor cells have round to oval vesicular nuclei, inconspicuous nucleolus, and moderate amount of pale eosinophilic cytoplasm (a and b). The cells are immunoreactive with CD56 (c, ×200), CD10 (d, ×200), cyclin D1 (e, ×200), and β-catenin (f, ×200)|
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| Discussion|| |
SPN was first described by Frantz in 1959. Approximately, 89% of the tumors occur in female with an age range of 7–79 years, however, majority of the patients are young. Though pancreas is affected in most of the cases, rare extrapancreatic cases (seven in the ovary, two each in the mesocolon, omentum, retroperitoneum, one each in the liver, stomach, and duodenum, respectively) have been reported.,,,, Ovarian SPNs are extremely uncommon. Patients present with abdominal pain, fullness, swelling, or may be asymptomatic, where the lesion is detected incidentally during imaging study for an unrelated reason. Radiologically, it can be solid-cystic or purely cystic. Our patient had abdominal pain and a nontender, solid-cystic adnexal mass. Microscopically, SPNs are low grade and display cord, trabecular and a characteristic pseudopapillary growth pattern with fibrovascular or myxoid cores lined by one or more layers of neoplastic cells with pale eosinophilic, vacuolated, or foamy cytoplasm. Nuclei are uniform with fine chromatin and indistinct or eccentrically placed small nucleolus. Microcystic spaces filled with eosinophilic colloid-like material and Periodic acid-Schiff positive intra- and or extra-cellular hyaline globules can also be seen. Extraovarian spread is unusual. In our case, cellular morphology was benign, but metastases were present in the multiple sites. Rarely SPN may show cellular atypia, perineural or angio-invasion, deep invasion into the surrounding tissue, indicating malignant behavior and some authors consider such lesion as solid-pseudopapillary carcinoma. Syriac et al. reported such a case in a 45-year-old female, who died of disease after 8 months of diagnosis. Morphologically benign appearing SPN, however, may metastasize as seen in our case. Because of this SPN is considered to be a malignant tumor. Though metastatic disease was present in our case at presentation, 18 months follow-up showed no tumor recurrence. The tumor cells are immunopositive with β-catenin, cyclin D1, CD56, CD10, vimentin, alpha antitrypsin, neuron specific enolase, and PR. Expression of synaptophysin and cytokeratin is variable, but there is a complete loss of E-cadherin. The present case also showed positivity with β-catenin, CD56, CD10, cyclin D1, and negativity with E-cadherin.
Differential diagnoses of ovarian SPN include granulosa cell/steroid cell tumor, struma ovarii, adenocarcinoma, and metastatic SPN. Granulosa cell tumor occurs during child-bearing age and in postmenopausal period and is associated with hyperestrinism in three-fourth cases. Characteristic Call-Exner bodies, longitudinal nuclear grooves, and immunoreactivity to vimentin, alpha inhibin, CD99, calretinin, and S100 are seen. Struma ovarii is characterized by >50% of thyroid tissue or an adenoma with follicles containing colloid. Steroid cell tumor usually occurs in postmenopausal women as a small solid, circumscribed mass of lutein cells which are positive for inhibin, calretinin, WT-1, and SF-1. In our case, no such features were seen and the pancreas was normal ruling out the possibility of metastasis.
The etiology of SPN is unknown, but female predominance and PR immunopositivity in most cases support the hypothesis of a hormonal role in the tumor development. PR, however, was negative in our case. β-catenin which serves as a structural component of E-cadherin-mediated cell-cell adhesion system and functions as a signaling molecule in the Wnt pathway is positive in almost all cases., Mutations of β-catenin gene in exon 3 have been found in 90% of SPNs.
Surgery is curative for localized disease and prolonged survival has been reported after adequate surgical resection in patients with metastatic disease. The role of chemotherapy is limited in unresectable tumors. Among the 17 cases of extrapancreatic SPNs including our case metastasis was seen in four patients, three of them had a long survival. Hence, recognition of such rare tumor is important for proper management of the patient and prediction of clinical outcome.
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Dr. Asit Ranjan Mridha
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]