A Curious Case of Autoimmune Pancreatitis with IgG4-related Sclerosing Cholangitis.

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a novel entity that belongs to the immune-mediated fibroinflammatory class of IgG4-related diseases (IgG4-RD). IgG4-SC is noted to be one of the most frequent manifestations of extra-pancreatic disease among IgG4-RD, which is significantly different from primary SC (PSC) and cholangiocarcinoma (CC) as is evident in the varied approaches to treatment. IgG4-RD includes IgG4-SC and autoimmune pancreatitis (AIP). Herein, we presented a case of IgG4-SC in a patient with obstructive jaundice secondary to AIP. We have also discussed the current recommendations for diagnostic and treatment modalities, with an emphasis on the issues that arise in obtaining a definitive classification of disease.

A Rare Case of Mixed Adenoneuroendocrine Carcinoma of the Ileocecal Valve.

Mixed adenoneuroendocrine carcinoma (MANEC) is an uncommon neoplasm with uncertain pathophysiology. In order to be classified as MANEC, the tumor must contain at least 30% neuroendocrine cells and 30% adenocarcinoma. The standardization of MANEC treatment has historically been difficult due to the lack of diagnostic histological classification. In 2010, the World Health Organization (WHO) finally recognized this uncommon condition as a specific colon cancer entity in the hopes of better specifying treatment options in the future. We present a case of high-grade MANEC of the cecum with metastasis in 3/10 lymph nodes to further characterize the diagnostic modalities and treatment options of the disease. MANECs only account for 3%-9.6% of all colorectal cancers and only eight cases have been reported in the cecum to date, making the following case report exceptionally rare.

Hereditary Colorectal Tumors: A Literature Review on MUTYH-Associated Polyposis.

MAP (MUTYH-associated polyposis) is a syndrome, described in 2002, which is associated with colorectal adenomas, with enhanced colorectal carcinogenesis. This review synthesizes the available literature on MAP and outlines its pathogenesis, association with colorectal tumorigenesis, screening, treatment, and the subtle differences between it and its close cousins-FAP and AFAP. The preponderance of data is collected using MAP guidelines. However, although AFAP and MAP appear similar, potentially important distinctions exist, warranting targeted diagnostic criteria and treatment approaches. We suggest that it may be prudent to screen for MAP earlier than in current clinical practice, as it has been shown that sequence variants are associated with more severe disease, presenting with an earlier onset of colorectal cancer. Finally, we issue a call-to-action for much-needed further data to establish clear clinical and diagnostic criteria.

A Rare Cause of Dysphagia to Remember: Calcific Tendinitis of the Longus Colli Muscle.

Longus colli tendinitis (LCT) is an acute inflammatory condition with symptoms typically consisting of acute neck pain and stiffness with or without dysphagia. Once more severe etiologies for these symptoms are ruled out, this self-limiting condition usually resolves spontaneously with nonsteroidal anti-inflammatory drugs and corticosteroids. We present a case of LCT that presented as acute neck pain, dysphagia, and odynophagia that rapidly resolved once diagnosed and treated with anti-inflammatory agents. Though exceedingly rare, LCT must be considered in the differential diagnosis of acute neck pain, dysphagia, and odynophagia when more common etiologies do not correlate with the clinical presentation.