IMAGE A man with peri-cecal, calcified mass—disseminated peritoneal adenomucinosis Francesco Guerra 1 & Danilo Coco 1 & Alberto Patriti 1 # Indian Society of Gastroenterology 2019 A 48-year-old, otherwise healthy male presented to the emer- gency department due to a fall from his bike. Abdominal ul- trasonography incidentally discovered a peri-cecal mass for which the patient was referred to the surgical department for further investigation. Upon physical examination, the abdomen was soft, non- tender, with normal bowel sounds. Routine blood investiga- tions were substantially normal apart from a slightly elevated C-reactive protein level of 1.1 mg/dL (upper limit 0.49). A contrast-enhanced computerized tomography (CT) scan of the abdomen revealed a hypoechoic peri-cecal mass measuring 11 × 4 × 3 cm. The mass showed enhanced, thick walls and sporadic central calcifications (Fig. 1, arrows). A plan for exploratory laparoscopy was made. Preoperative laboratory tests showed a serum carcinoembryonic antigen (CEA) level of 4.8 ng/mL (normal range 0–5.0), whereas serum cancer antigen 19-9 (CA 19-9) was 70.6 U/mL (0–35.4). At surgery, a scarce amount of ascites was present, and multiple fibro-gelatinous peritoneal nodules were encountered (Fig. 1a). A bulky appendix displacing the cecum and ascend- ing colon medially was also noticed (Fig. 1b). Pathologic ex- amination of peritoneal biopsies revealed its involvement by the mucinous neoplasm, with paucicellular, abundant mucin pools (Fig. 2a) and scant strips of low-grade neoplastic epi- thelium (Fig. 2b, c). The patient was diagnosed having disseminated peritoneal adenomucinosis (DPAM) and scheduled to receive cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). DPAM or “pseudomyxoma peritonei” (PMP) indicates a clinical syndrome in which the peritoneal cavity is studded with mucus-producing implants originating from ruptured appendiceal mucinous epithelial neo- plasms [1, 2]. A peripheral pattern of tumor dissemination with relative sparing of small bowel and mesentery (the so-called redistribution phenomenon) and the thickening of the undersur- face of the diaphragm by multiple mucinous nodules are char- acteristic (Fig. 1a)[3]. The imaging diagnosis of PMP essentially relies on the iden- tification of the mucocele and the intra-abdominal spread of mu- cin [4, 5]. Ultrasonography findings usually include a sausage- or round/oval-shaped cystic mass with variable internal echogenicities in the expected area of the appendix. Most lesions present thin inner echogenic rim and outer echolucent layer, representing bowel wall. Internal septae, intramural calcifica- tions, and signs of intussusception may also be present. Echogenic peritoneal masses or ascites with not-moving echogenic particles are also typical. CT usually confirms curvi- linear or punctate calcifications and commonly reveals good, homogeneous contrast enhancement of the wall of the mucocele, while the presence of intramural focal enhancing nodules is often a sign of high-grade peritoneal mucinous carcinomatosis (PMCA). Common extra-appendiceal features include the pres- ence of simple or loculated low-attenuation intra-peritoneal fluid, omental caking, and serosal implants on solid organs, producing a scalloped appearance on their surfaces [4, 5]. Differential diag- nosis of an appendiceal mucocele includes, among others, appendicitis-related abscesses, hydrosalpinx, lymphocele, and mesenteric or enteric duplication cysts. The identification of in- tramural calcifications, and an echogenic rim consistent with a lining epithelium are usually sufficient to exclude most mimick- ing diseases [4]. Because of the presence of the extra-appendiceal manifestations, other intraperitoneal diseases, such as primary peritoneal mesothelioma, abdominal tuberculosis, septic perito- nitis, and secondary peritoneal carcinomatosis, should be also * Francesco Guerra fra.guerra.mail@gmail.com 1 Ospedali Riuniti Marche Nord, Pesaro, Italy Indian Journal of Gastroenterology https://doi.org/10.1007/s12664-019-00982-3