Pseudomyxoma Peritonei Revisited: A Case Report

Bleeding after menopause raises suspicion of malignancy; more so, if combined with increased abdominal girth and constitutional symptoms. This is a case of a 74-year-old Gravida 10 Para 8 (8026) who presented with generalized abdominal pain, enlargement, bloatedness and vaginal bleeding. Ultrasound revealed a complex abdominopelvic mass, likely ovarian in origin. Tumor markers CA-125 and CA-199 were elevated. Endometrial curettage with frozen section revealed Leiomyosarcoma. It was followed by exploratory laparotomy revealing gelatinous material in the peritoneum with seeding of mucoid material into the omentum, ovary and appendix. Frozen section of the right ovary revealed Atypical Mucinous Proliferative Ovarian Tumor (APMOT). Final histopathology result of the endometrial curetting revealed adenomatoid tumor of the uterus. Immunohistochemical staining with desmin and caldesmon revealed negative results implicating the absence of leiomyosarcoma. Final histopathology results were consistent with Disseminated Peritoneal Adenomucinosis (DPAM). Immunohistochemical staining with CK20 was positive and CK7 was negative, consistent with metastases from a primary gastrointestinal tumor. Chemotherapy in the form of FOLFOX regimen was contemplated. However, the patient was lost to follow up.

Progression of peritoneal adenomucinosis to the scrotum: a rare occurrence treated with cytoreductive surgery and hyperthermic chemoperfusion of the scrotum in two patients

Introduction: Disseminated Peritoneal Adenomucinosis (DPAM) is an infrequent presentation of appendiceal cancer. Infrequently, umbilical or inguinal hernias could be the first clinical manifestation of this condition; DPAM extension to the scrotum may be anatomically viable. Treatment with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard of treatment for DPAM. We hypothesize that these same treatment principles, consisting of CRS with hyperthermic chemoperfusion of the scrotum (HCS), could be applied to the scrotal dissemination of DPAM. Methods: We reviewed our Institution’s prospective cancer database and identified two cases of DPAM with extension to the scrotum. Their medical records were examined, and close follow-up was performed. Tumor histopathology and cytoreduction scores were evaluated. Tumor progression was monitored on follow-up by physical examination, tumor markers (CEA, CA 125, CA 19.9) and abdomino-pelvic CT scan. Results: Two patients who previously had CRS/ HIPEC for DPAM were successfully treated with HSC. Both patients are alive and free of disease at 88 and 57 months following initial CRS/HIPEC, and 50 and 32 months following CRS/HCS, respectively. Conclusion: Increased awareness by surgeons to the coexistence of inguinal hernia with peritoneal neoplasm and the need for a surgical repair is raised. CRS/HCS may be employed to treat patients with DPAM extension to the scrotum. Successful outcome is dependent on complete cytoreduction of metastatic tumor.

Pseudomyxoma Peritonei Originating from an Intestinal Duplication

Alimentary tract duplications are rare congenital anomalies. They most often become symptomatic in childhood and rarely undergo malignant transformation. Pseudomyxoma peritonei (PMP) is an equally uncommon condition, most frequently originating from a primary appendiceal mucinous neoplasm. We report an extremely unusual case of PMP arising from an intestinal duplication. A 67-year-old woman presented with vague upper abdominal pain, and, unexpectedly, explorative laparoscopy revealed diffuse jelly-like peritoneal implants. The histopathological diagnosis of a low-grade PMP or “disseminated peritoneal adenomucinosis” was made. At that moment, no primary tumor was found. During later surgery, a cystic lesion located in the mesentery of the small bowel could be resected. Histologically, the cyst wall clearly showed the concentric layering of a normal bowel wall. The mucosa, however, displayed a diffuse low-grade villous adenoma. We concluded that this histological picture was most consistent with a small intestinal duplication, containing a low-grade villous adenoma. The adenoma caused a mucocele, which subsequently leaked or ruptured, giving rise to noninvasive mucinous peritoneal implants or low-grade PMP, also known as “disseminated peritoneal adenomucinosis” (DPAM).