Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.

Malignant Transformation of Ectopic Pancreas.

Heterotopic pancreas (HP) can be detected by accompanying symptoms or incidentally during gastrointestinal (GI) tract tumor resection. We compared clinicopathologic features among 165 resected HPs (57 gastric [35%], 56 duodenal [34%], 30 omental [18%], and 22 jejunal [13%]). Symptomatic HPs (79/135 GI tract wall HPs, 59%) were larger (P=0.05), more common in younger patients and in a gastric location (both P<0.001), and more frequently associated with lymphoid cuffs (P=0.03) than incidentally found HPs. Gastric/jejunal HPs were more frequently symptomatic (P<0.001), deeply located (P=0.03), and associated with lymphoid cuffs (P=0.008) and pancreatic intraepithelial neoplasia/intraductal papillary mucinous neoplasms (PanIN/IPMN; P=0.001) than duodenal HPs. HP was frequently associated with acinar-ductal metaplasias (117/135 GI tract wall HPs, 87%) and PanINs/IPMNs (68/135, 50%); those with PanINs/IPMNs were larger (P<0.001), more frequently located in stomach (P=0.001), had deeper wall involvement (P=0.03), and more often showed infiltrative growth (P<0.001) and lymphoid cuffs (P=0.02). Four HPs containing PanINs abutted adenocarcinomas, all expressing wild-type KRAS and intact SMAD4/DPC4 expression. Thus, symptomatic HP is associated with younger age, larger size, gastric location, and lymphoid cuffs. HPs containing PanINs/IPMNs (usually low grade) are larger and more common in stomach, have deeper wall location, and show infiltrative growth and lymphoid cuffs. Adenocarcinomas are rarely observed adjacent to HPs with PanINs/IPMNs. KRAS mutational and SMAD4/DPC4 immunohistochemical studies can discriminate between adenocarcinoma derived from HP and concurrent adenocarcinoma with HP.

Heterotopic Pancreas of the Gastrointestinal Tract and Associated Precursor and Cancerous Lesions: Systematic Pathologic Studies of 165 Cases.

A 62-year-old Japanese man presented to our hospital with a history of weight loss of 6 kg in 4 mo. Imaging examinations revealed a tumor located on the third portion of the duodenum with stenosis. We suspected duodenal carcinoma and performed pancreas-preserving segmental duodenectomy. Adenocarcinoma arising from a heterotopic pancreas at the third portion of the duodenum was finally diagnosed by immunohistochemical staining. Malignant transformation in the duodenum arising from a heterotopic pancreas is extremely rare; to our knowledge, only 13 cases have been reported worldwide, including the present case. The most common location of malignancy is the proximal duodenum at the first and descending portion. Herein, we describe the first case of adenocarcinoma arising from a heterotopic pancreas, which was located in the third portion of the duodenum, with a review of the literature.

Adenocarcinoma arising from heterotopic pancreas at the third portion of the duodenum.

BACKGROUND Heterotopic pancreas (HP) is an aberrant anatomic malformation that occurs most commonly in the upper gastrointestinal tract. While the majority of heterotopic pancreatic lesions are asymptomatic, many manifest severe clinical symptoms which require surgical or endoscopic intervention. Understanding of the clinical manifestations and symptoms of HP is limited due to the lack of large volume studies in the literature. The purpose of this study is to review symptomatic cases at a single center and compare these to a systematic review of the literature in order to characterize common clinical manifestations and treatment of this disease. AIM To classify the common clinical manifestations of heterotopic pancreas. METHODS A retrospective review was conducted of pathologic samples containing heterotopic pancreas from 2000-2018. Review was limited to HP of the upper gastrointestinal tract due to the frequency of presentation in this location. Symptomatic patients were identified from review of the medical records and clinical symptoms were tabulated. These were compared to a systematic review of the literature utilizing PubMed and Embase searches for papers pertaining to heterotopic pancreas. Publications describing symptomatic presentation of HP were selected for review. Information including demographics, symptoms, presentation and treatment were compiled and analyzed. RESULTS Twenty-nine patient were identified with HP at a single center, with six of these identified has having clinical symptoms. Clinical manifestations included, gastrointestinal bleeding, gastric ulceration with/without perforation, pancreatitis, and gastric outlet obstruction. Systemic review of the literature yielded 232 publications detailing symptomatic cases with only 20 studies describing ten or more patients. Single and multi-patient studies were combined to form a cohort of 934 symptomatic patients. The majority of patients presented with abdominal pain (67%) combined with one of the following clinical categories: (1) Dyspepsia, (n = 445, 48%); (2) Pancreatitis (n = 260, 28%); (3) Gastrointestinal bleeding (n = 80, 9%); and (4) Gastric outlet obstruction (n = 80, 9%). The majority of cases (n = 832, 90%) underwent surgical or endoscopic resection with 85% reporting resolution or improvement in their symptoms. CONCLUSION Heterotopic pancreas can cause significant clinical symptoms in the upper gastrointestinal tract. Better understanding and classification of this disease may result in more accurate identification and treatment of this malformation.

Clinical classification of symptomatic heterotopic pancreas of the stomach and duodenum: A case series and systematic literature review

The malignant transformation of an ectopic pancreas in the duodenum is extremely rare. Herein, we report a case of an adenocarcinoma that arose from an ectopic pancreas. We also reviewed 14 cases of malignant transformations arising from an ectopic pancreas in the duodenum that were previously published. An 81-year-old man with a 1-month history of vomiting was admitted to our institution. Esophagogastroduodenoscopy (EGD) and computed tomography (CT) scans revealed an obstruction at the first part of the duodenum. A distal gastrectomy was performed for diagnostic and therapeutic purposes. The histopathological examination of the resected specimen showed adenocarcinoma that arose from an ectopic pancreas (Heinrich type 1). The patient is alive without relapse at 18 months of follow-up. Adenocarcinoma that arises from an ectopic pancreas should be considered when an obstruction is identified in the duodenum.

/pdf/adenocarcinoma-arising-from-an-ectopic-pancreas-in-the-1lzao006aq.pdf

Adenocarcinoma arising from an ectopic pancreas in the duodenum: a case report.

Ectopic pancreas is defined as pancreatic tissue outside the normal location without connection to the normal pancreas. It occurs throughout the gastrointestinal tract, most commonly in the stomach (25-60%), followed by the duodenum (25-35%) and jejunum (16%). It may develop the same pathological changes of a normal pancreas such as acute pancreatitis and cyst formation. Malignant degeneration rarely occurs. We present a case of heterotopic pancreatic adenocarcinoma localized in the duodenal bulb presenting with symptoms of gastric obstruction.

Pancreatic adenocarcinoma in duodenal ectopic pancreas: a case report and review of the literature.

Tubulo-squamous polyp is an infrequent, apparently benign lesion which has recently been described in the vagina. Histologically, it is characterized by an epithelial component, consisting of squamous nests and tubules, embedded in a hypocellular fibrous stroma. Here, we report an unusual histological variant showing a cellular stroma, as an additional histologic feature, which could lead to confusion with other site-specific lesions of the vagina. A brief review of the literature on this entity is also provided.

Tubulo-squamous polyp of the vagina. A case with cellular, "angiomyofibroblastic-like" stroma.

Pleomorphic ductal invasive carcinoma is a very rare, high-grade breast cancer with unfavourable prognosis. It contains highly pleomorphic giant cells, which represent more than 50% of the cancer cells. One such case is described herein, focusing on its morphological, histopathological and immunohistochemical patterns. It was multicentric, oestrogen and progesterone receptor negative and epidermal growth factor receptor type 2 positive.

Pleomorphic giant cell ductal carcinoma of the breast.

We report the first case of a patient affected by peritoneal metastases from colon cancer, arising in the context of Lynch syndrome with pathological complete response. The patient was treated with immunotherapy and cytoreductive surgery. This paper discusses the implications of these novel therapies for the management of PM.A 50-year-old man affected by Lynch syndrome was referred to our institution for metachronous peritoneal recurrence of ascending colon adenocarcinoma. As a second-line treatment, he received Nivolumab therapy with stable disease. Patient underwent cytoreductive surgery with residual disease and a pathological complete response. Flow cytometry described a particular immune sub-population response. There was no evidence of disease progression after nine months.This is the first report of a Lynch patient affected by peritoneal metastases of colorectal cancer, treated with cytoreductive surgery (CRS) and resulting in a pathological complete response after immune checkpoint inhibitors treatment (ICIs). This case report may suggest that patients with peculiar immunological features could benefit from a tailored approach, since "classical" CRS paradigms may not effectively predict the clinical outcome. Further large-scale studies are needed to determine the correct operative management of such patients (tailored or "standard" CRS), defining the correct surgical timing and eventual discontinuation of ICI therapy after surgery. 

/pdf/complete-pathological-response-of-colorectal-peritoneal-1fhcfbsg.pdf

Complete pathological response of colorectal peritoneal metastases in Lynch syndrome after immunotherapy case report: is a paradigm shift in cytoreductive surgery needed?

Background Paratesticular fibrous pseudotumor (PFP) is a rare intrascrotal benign fibrous mass of uncertain aetiology, usually arising between testicular tunica layers and is supposed to be related to inflammatory reactive conditions. Because of morphological similarities to IgG4-related sclerosing fibro-inflammatory lesions, some authors recently postulated that PFP might belong to the IgG4-related disease (IgG4-RD) family. Considering the rarity of this lesion, only few cases have been reported in literature about the correlation between IgG4-RD and PFP. Management of PFP could be extremely challenging: due to the lack of typical clinical signs and the non-specific radiological characteristics, misapprehension does occur in the majority of cases, mainly because these intrascrotal mass may mimic testicular neoplasm, therefore leading to radical orchidectomy rather than a desirable testis-sparing surgery. Case Description Herein we report two cases of young males treated for PFP with histological feature of IgG4-RD. Patients underwent testicular sparing surgery. At 2-year follow-up no evidence of local or distant relapse nor testicular disorder was observed in both patients. An up-to-date review of the literature about the correlation between PFP and the IgG4-RD was carried out. Conclusions PFP is an extremely rare condition with uncertain etiology being part of IgG4-RD family. Preoperative imaging mimics malignancy hence diagnosis is usually made by specimen analysis. Intraoperative frozen section is fundamental in order to guarantee conservative treatment that is feasible and safe after mid-term follow-up.

Paratesticular fibrous pseudotumor with histological features of IgG4-related disease: two case reports and review of the literature

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