Fatal sclerosing mesenteritis: a 7-year-old male autopsy case report.

Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

Non-Cirrhotic Portal Hypertension in Systemic Lupus Erythematosus.

We report the case of idiopathic non-cirrhotic portal hypertension associated with systemic lupus erythematosus in a 43-year-old woman who suffered from breast cancer. We review this rare condition, as well as its diagnostic and therapeutic approaches.

Fatal sclerosing mesenteritis: a 7-year-old male autopsy case report

ABSTRACT Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

Endometriosis-associated intestinal tumors: a new challenge in clinical practice.

A 66-year-old female consulted due to headache associated with diaphoresis, constipation, and melena. A urinalysis revealed elevated metanephrines, and colonoscopy identified an extrinsic sigmoid stricture. CT and MRI showed a presacral mass infiltrating the sigma. Scintigraphy and SPECT ruled out a diagnosis of paraganglioma. Based on these findings, the patient was proposed to undergo surgery.

Mucinous adenocarcinoma arising from chronic perianal fistula-a multidisciplinary approach.

Mucinous adenocarcinoma (MA) is a rare entity. Indeed, the pathogenesis of fistula-associated perianal MA is still controversial. Due to the lack of informed evidence regarding this malignancy, no guidelines have been established concerning diagnostic and treatment strategies. The aim of this article is to report our experience and outcomes after three cases of large perianal MA treated in our center. From our retrospective chart review, we identified three male patients with chronic perianal fistula-in-ano who progressively developed perianal MA, confirmed by pelvic magnetic resonance (MRI) and histopathological examination performed on biopsy. We hereby, in accordance with the Surgical CAse REport (SCARE) guidelines, describe the management and further follow-up of each patient. The three patients underwent preoperative chemoradiation therapy, followed by ischioanal abdominoperineal resection (APR). Perineal reconstruction was needed in every case, using a vertical rectus abdominis myocutaneous (VRAM) flap and, punctually, a left fasciocutaneous flap was used too. Also, two of three patients completed the treatment with adjuvant chemotherapy. Neither recurrences nor distant metastases have been observed during the follow-up in both cases that finished the multimodal treatment. MA arising from chronic perianal fistula has an indolent growth with locoregional aggressiveness and a high risk of local recurrence. Therefore, although an ischioanal APR remains the surgical treatment of choice, an aggressive multimodal approach combining preoperative chemoradiation and adjuvant chemotherapy may achieve favorable effectiveness and promising response rates.

Primary squamous cell carcinoma of the stomach: a rare entity

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Primary squamous cell carcinoma of the stomach: a rare entity.

We present the case of a primary squamous cell carcinoma (PSCC) and inform about the evidence to date and also highlight the diagnostic criteria. We present the case report of a 78-year-old female was admitted due to a 2-week history of abdominal pain, weight loss and vomiting.