A 49-year-old woman, who had been suffering for some time from irritable bowel syndrome (IBS), presented to the emergency department complaining of pain in the right lower quadrant (RLQ). She reported a two-week history of constipation and pain in the RLQ and a temperature of 38.5°C. Over the last two days, she had experienced pain at defecation, tenesmus, rectal bleeding and episodes of hematuria. The condition showed no improvement with antispastic therapy or pain killers. Her medical history included IUD insertion/retrieval and two uncomplicated deliveries, while her family history included colonic adenocarcinoma in her mother. The blood test pointed out leukocytosis and elevated concentration of CRP.
At the medical examination a flat, sore abdomen in the RLQ and the presence of a deep stiff mass with undefined borders were observed. CT scan pointed out an appendix rising postero-medially to lumbosacral level for about 10 cm; it was markedly thickened and patchy, with increased density of the surrounding fat tissue consistent with a phlegmon (Figure 1). Periureteral tissue was affected by the inflammatory reaction. Abdomino-pelvic lymph nodes were slightly enlarged, in particular at the mesenteric periappendicular level, the axial size rising to 20 mm. Given the clinical presentation and radiological findings, the patient underwent a diagnostic laparoscopy. A bent, phlegmonous appendix, 6.5 cm in length and with a swollen tip, was noted enclosed within an inflammatory plastron; it adhered tenaciously to the last ileal loop (at the caecal fundus) and to the right fallopian tube fimbria. A laparoscopic appendectomy was performed with a careful management, isolating the appendix and separating it from the tube and the thickened last ileal loop. A stiff greyish right ovarian lesion of 3.7 × 3.8 cm was also found; the left ovary had no significant macroscopic changes.
The surgical specimens were fixed in 10% neutral buffered formalin and then paraffin embedded. Besides to haematoxylin-eosin, histochemistry (Luxol fast blue, Pas-diastase) and immunohistochemistry (Ki-67, p53, S100, NSE, calretinin) were performed, applying the standard avidin-biotin complex (ABC) method.
Specimens examination revealed a florid granulomatous chronic inflammation with foreign body-type multinucleated giant cells and mastocytes (Figure 2) in the swollen tip of the appendix. Adjacent to this, a submucosal GCT nodule was present, 0.2 cm in diameter (Figures 2, 3 and 4). The tumor was uncapsulated, well circumscribed and showed no trend to infiltration; it was devoid of necrosis and cytological atypia and showed a low cytoproliferative activity (MIB1-LI). The tumor cells resulted immunoreactive for S-100 protein, neuron-specific enolase (NSE) and calretinin; p53 was not detected. The cytoplasmatic granules were ascertained by Luxol fast blue and Pas-diastase stainings. Above the tip, the phlogosis was acutely exacerbated. There was a prevalence of neutrophils and fibrin in relation to a perforation of the appendix wall and a reactive septated peritonitis. The ovarian specimen proved to be a classic leiomyoma (fibroma) without cell atypia; the fallopian tubes were slightly atrophic. The postoperative phase was uneventful and the patient was discharged on postoperative day four. An integrated PET-CT scan performed one month after hospital discharge revealed no signs of recurrence. In view of the histopathological diagnosis of benignancy and family history of colonic cancer, the oncologist suggested a follow-up with colonoscopy every 3 years. In literature no data are reported, which correlate the GCT incidence with the risk for colorectal cancer, and our effort has been aimed in the clinical management of patients affected by GCT involving the lymphatic tissue-rich sites, such as ileo cecal appendix.