Multiple endocrine neoplasia type 1 (MEN1), an autosomal-dominantly inherited tumor syndrome, is classically defined by tumors arising from the “3 Ps”: Parathyroids, Pituitary, and the endocrine Pancreas. From its earliest descriptions, MEN1 has been associated with other endocrine and non-endocrine neoplastic manifestations. High quality evidence supports a direct association between pathogenic MEN1 variants and neoplasms of the skin (angiofibromas and collagenomas), adipose tissue (lipomas and hibernomas), and smooth muscle (leiomyomas). Although CNS tumors, melanoma, and, most recently, breast cancer have been reported as MEN1 clinical manifestations, the published evidence to date is not yet sufficient to establish causality. Well-designed, multicenter prospective studies will help us to understand better the relationship of these tumors to MEN1, in addition to verifying the true prevalence and penetrance of the well-documented neoplastic associations. Nevertheless, patients affected by MEN1 should be aware of these non-endocrine manifestations, and providers should be encouraged always to think beyond the “3 Ps” when treating an MEN1 patient.

/pdf/beyond-the-3-ps-a-critical-appraisal-of-the-non-endocrine-2nzh9r6l.pdf

Beyond the “3 Ps”: A critical appraisal of the non-endocrine manifestations of multiple endocrine neoplasia type 1

Colonic leiomyomatous lesions are smooth muscle tumors including both benign leiomyoma and malignant leiomyosarcoma. They usually occur in elderly and middle-age groups. They are rare representing 3% of all gastrointestinal leiomyomas. Descending and sigmoid colon are the most common affected segments of colon. Patients are usually asymptomatic but occasionally they may present with abdominal pain, hemorrhage, and intestinal obstruction. Radiological findings for these lesions are variable and overlaping with other diagnosis but usually manifest as large lesions with lobulated margins, extra-colic growth and heterogenous enhancement. Final diagnosis is achieved by tissue diagnosis in which immunohistochemistry is used to differentiate them from other types of mesenchymal tumors like Gastrointestinal Stromal Tumor (GIST). A complete surgical/endoscopic resection is usually curative with excellent prognosis in cases of benign leiomyoma. In cases of leiomyosarcoma, post-surgical chemotherapy and radiotherapy is usually needed with five-year survival of about 50%. We report the radiological findings of two cases of colonic leiomyomatous lesions that presented with nonspecific abdominal pain. The lesions were resected surgically and confirmed histopathologically as leiomyoma and leiomyosarcoma of the colon, respectively. We report the radiological findings with pathological correlation and literature review. To the best of our knowledge, these are the first cases of colonic leiomyomatous lesions to be reported in our country.

Leiomyomatous Lesions of the Colon: Two Case Reports with Radiological Features, Pathological Correlations and Literature Review

Gastrointestinal stromal tumour (GIST) is the most common mesenchymal neoplasm arising in the stomach. However, a number of other rare mesenchymal neoplasms do occur at this anatomic site, which often presents a diagnostic challenge for cytopathologists on endoscopic ultrasound‐guided fine‐needle aspiration (EUS‐FNA). Our study aims to selectively present the clinico‐radiological and cytopathological characteristics of these rare “non‐GIST” neoplasms, as well as their differential diagnoses.

Cytopathology of rare gastric mesenchymal neoplasms: A series of 25 cases and review of literature

Leiomyoma is a smooth muscle tumour that can arise in any part of the body especially the uterus. Even though it is traditionally linked with hormonal inf luence, it can also develop in extrauterine organs with a slight female predominance. It is indistinguishable with gastrointestinal stromal tumour (GIST) histologically. We report a case of a 30-year-old gentleman who presented with a huge painful mass in the right iliac fossa. Computed tomography revealed a 10 × 10 cm homogeneous mass arising from the terminal ileum; he subsequently underwent an open right hemicolectomy. Histology showed a well-circumscribed lesion composed of interlacing bundles of smooth muscle fibres of the submucosa with positive smooth muscle actin and H-Caldesmon stains but negative for DOG-1 and CD117 (c-kit) stains which were consistent with leiomyoma. Despite its rarity, this hormone-related tumour needs to be considered regardless of gender. Immunohistochemistry is paramount as it is histologically identical to GIST.

An oncological curiosity of a male patient with a huge leiomyoma of the terminal ileum.

Sarcomas are rare lesions and encompass a wide variety of entities, depending on their nature. In recent years new entities have been described and new knowledge, especially that provided by molecular studies, has been increasing. This makes it very difficult to be updated with all the described entities, since only some of the centers have the desired ancillary studies for the correct diagnosis. Some lesions are extremely rare and may appear once or twice during the lifetime of a general pathologist. When we refer to sarcomas of the gastrointestinal tract, the gastrointestinal stromal tumor (GIST) is the most well-known lesion that the pathologist will most frequently find in daily practice. This paper aims to comprehensively review the sarcomas associated with the gastrointestinal tract, emphasizing histopathology and going beyond GIST. This review highlights the histopathology of rare types of sarcomas so it may increase awareness of common and rare lesions, prompting an easy and effective diagnosis.

Mesenchymal Tumors of the Gastrointestinal Tract—Beyond GIST—A Review

Smooth muscle tumors of the gastrointestinal tract: an analysis of prognostic features in 407 cases

Unique presentation of a gastrointestinal stromal tumor, epithelioid type, with a dual copy loss of SDHA gene as a liver primary

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Smooth muscle tumors of the gastrointestinal tract: an analysis of prognostic features in 407 cases

Unique presentation of a gastrointestinal stromal tumor, epithelioid type, with a dual copy loss of SDHA gene as a liver primary