|Year : 2016 | Volume
| Issue : 1 | Page : 140-142
Pseudomyxoma peritonei in a young male
Pavani Marapaka1, Jayashankar Erukkambattu1, Satyanarayana Gorthi2, Ramamurti Tanikella1
1 Department of Pathology, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India
2 Department of General Surgery, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India
|Date of Web Publication||22-Dec-2015|
Plot No. 176, Christian Colony, Ranga Reddy, Hyderabad - 500 070, Telangana
Source of Support: None, Conflict of Interest: None
Pseudomyxoma peritonei (PMP) is an uncommon surgical entity, characterized by diffuse deposition of mucin within the peritoneal cavity secondary to mucinous tumors of the ovary or appendix. This gelatinous ascites or "jelly-belly" may result in death from loss of intestinal function and intestinal obstruction. Preoperative diagnosis on cytology is rare. Herein, we report a case of PMP in a young male who presented with intestinal obstruction, diagnosed preoperatively on cytological examination, and confirmed later by histopathological examination.
Keywords: Cytology, intestinal obstruction, pseudomyxoma peritonei
|How to cite this article:|
Marapaka P, Erukkambattu J, Gorthi S, Tanikella R. Pseudomyxoma peritonei in a young male
. Med J DY Patil Univ 2016;9:140-2
| Introduction|| |
Pseudomyxoma peritonei (PMP) is a rare disease, with an overall incidence of 1-2 per million per year.  Werth was the first to describe this entity in 1884.  PMP is a clinical term for diffuse intra-abdominal gelatinous collections ("jelly-belly") with mucinous implants on the omentum and peritoneal surface and is three to four times more common in women.  Patients usually present with abdominal distension, mucinous ascites ("jelly-belly") and with intestinal obstruction in advanced stages.  Site of origin is thought to be appendix in men and ovaries in females. We here in describe the rare cytodiagnosis of PMP given in a young male.
| Case Report|| |
A 33-year-old young man presented to our surgical department with complaints of pain abdomen and vomiting since 1-week. On examination, he had abdominal distension, tenderness and a vague mass palpable in the right iliac fossa (RIF). No weight loss or comorbidities noted. With the clinical diagnosis of acute intestinal obstruction, the patient was evaluated with ultrasonogram and computed tomography of abdomen, which revealed a soft tissue mass in the RIF, diffuse omental thickening nodules, and moderate ascites.
The differential diagnosis on radiology included:
On microscopic examination, the smears showed mucin pools, a few clusters/monolayered sheets of atypical cells admixed with benign sheets of mesothelial cells. These cells are round to oval, with well-defined cell borders, round to oval hyperchromatic nuclei with moderate to abundant cytoplasm. No mitotic figures seen [Figure 1]. Impression was given as PMP.
- Peritoneal carcinomatosis,
- Gastrointestinal stromal tumor, and
- Ruptured mucocele of the appendix. Guided fine needle aspiration from the mass was done. Aspirate was thick and gelatinous, and sent for cytological examination.
|Figure 1: (a) Smears showing mucinous background (H and E, ×100) (b) Mucin pools and cells floating in the mucin (H and E, ×100). (c) A sheet of atypical cells in the mucinous background (H and E, ×100)|
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With this diagnosis, the patient underwent Sugarbaker and Chang  procedure. Intra-operatively, the patient had extensive surface deposits on the peritoneum, whole of colon, spleen, liver, gallbladder and ileum. No parenchymal involvement noted [Figure 2]a-d]. Minimal ascites present.
|Figure 2: (a) Omental deposits (b) gallbladder (c) spleen (d) colectomy specimen along with part of ileum. (e and f) Histopathology — glands lined by single layer of cells and cells floating in mucin pools (H and E,|
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We received intra operative ascetic fluid for cytology and whole of the colon except for a part of sigmoid colon, gallbladder, and spleen. Histopathology of the appendix revealed extensive thick and thin mucinous areas, glands lined by single layered columnar cells with intervening goblet cells and mild pleomorphism. Stromal invasion was not evident [Figure 2]e and f].
The microscopic findings were similar in all the surgically resected organs. Only surface involvement was present and parenchyma was free. Final impression was given as low-grade mucinous neoplasm of the appendix with extensive PMP and confirmed the diagnosis of PMP given on cytology.
Microscopic examination of the ascitic fluid showed mucoid background with tumor cells arranged predominantly in single cells and occasional clusters with the acinar arrangement. These cells have round to oval nuclei with mild pleomorphism, hyperchromasia and moderate to the abundant eosinophilic cytoplasm, and the report was given as malignant effusion [Figure 3].
|Figure 3: (a) Cellular smears against mucinous background (H and E, ×40). (b) Cells in acinar pattern admixed with mesothelial cells (H and E, ×400)|
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| Discussion|| |
Pseudomyxoma peritonei is an indolent disease and preferentially affects women with an average age of 53 years.  Here, we report a case of a young male with a preoperative diagnosis of PMP, made on cytology.
Patients with advanced stage will manifest multiple adhesions and can present with intestinal obstruction  as we had noted in our case. The another reason for acute intestinal obstruction in case of PMP may be due to multiple surgeries.  In the present case, the patient did not undergo any surgeries previously. The diagnosis of PMP requires the presence of neoplastic cells in the mucin and diffuse intra-abdominal mucin.  Cytology of our case revealed mucin pools, and epithelial cells in clusters, sheets and acinar pattern.
Appendix and ovary are thought to be the sites of origin in male and females respectively, but recent evidence suggests that even in females appendix is the site of origin.  The pathogenesis of PMP is attributed to multifocal neoplasia of the peritoneum, ovaries, and appendix, mucinous metaplasia of the peritoneum, metastasis, rupture, or leakage from a primary mucinous neoplasm.  In the present case low-grade mucinous lesion of the appendix is the site of origin.
Ronnett et al.,  prognostically classified PMP into three types based on cytomorphological features:
Shin and Sneige  prognosticated PMP based on number of malignant epithelial cells in ascitic fluid into +1 (rare), to +3 (many). Jackson et al.  in their study contradicted this and proposed that the cytological classification as DPAM or PMCA based on cellular features indicates prognosis. In our case, we observed multiple clusters of epithelial cells with mild to moderate atypia.
- Disseminated peritoneal adenomucinosis (DPAM),
- Peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D) and
- PMCA. The cytomorphologic features for DPAM are cohesive clusters or monolayered sheets of cells with uniform regular round nuclei and without any mitotic figures. Our case showed features similar to DPAM.
Sugarbaker and Chang  recommended that patients with mucinous neoplasm and PMP be treated with extensive cytoreduction followed by intra and postoperative chemotherapy. Our case had maximum cytoreduction with Sugarbaker surgery. The patient is on regular review in outpatient clinic and remains asymptomatic.
| Conclusion|| |
Cytological diagnosis of PMP is rare. Preoperative diagnosis of PMP on aspiration cytology will help in patient management by allowing the surgeon to perform appropriate debulking cytoreductive surgery.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]