Presentation
Right lower abdominal pain and fullness since 2 to 3 months
Patient Data
An irregular large predominantly cystic lesion with central calcifications and air is noted in the right iliac fossa, representing appendiceal mucinous neoplasm with moderate ascites.
There is scalloping of liver surfaces representing pseudomyxoma peritonei.
Few loco-regional lymph nodes are noted.
Rest of the organs are normal. No intestinal obstruction. No metastasis.
Case Discussion
Histopathology report :
These needle core samples show pools of mucin with small clusters of malignant cells.
Focally the mucin is dissecting into tissues with surrounding fibrous reaction. There is extra and intracellular mucin.
On IHC, the tumor cells show positive staining with CDX2 and CEA. CK20 shows focal patchy staining. No staining is seen with CK7, WT-1, GATA-3, PAX-8, Vimentin, CK19, TTF-1, CA125 and ER.
The overall morphological and immunohistochemical features are in keeping with Mucinous neoplasm favoring a GI primary however other primary sites cannot be excluded. Accurate grading is not possible on biopsy sample. Please correlate with the radiological findings.
Mucin producing tumors of the appendix are the most common cause of pseudomyxoma peritonei. Premalignant tumors are usually treated by surgical excision and malignant lesions by right hemicolectomy.
Pseudomyxoma peritonei is treated by cyto-reductive surgery and intraperitoneal chemotherapy.
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