Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, which typically have a perivascular location with dual melanocytic and muscular differentiation. They are found in a variety of localizations, though lesions in the liver are exceedingly rare. Because of their rarity, the clinical, radiological and histological features of these tumors have yet to be established. This is why, it seems appropriate to report the observation of this rare hepatic tumor with a literary review including others published cases, assessing through it, clinicopathologic and radiologic features of all reported cases as well as their follow-up whenever possible.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1967094999126169

BackgroundColorectal Cancers (CRC) are one of the most common malignancies in the world. Their incidence in Morocco, between 2005 and 2007, was 5.6 for 100000 inhabitants, which is very low compared to what found in developed countries. In addition, CRCs show a high frequency of rectal localizations, and occurs in a younger population in Morocco compared to what found in developed countries.The purpose of this study is to confirm these CRC peculiarities in Morocco and try to explain them by exploring the microsatellite instability molecular pathway.MethodsThis is a prospective observational study conducted since January 2010, including 385 patients admitted in Hassan II University Hospital of Fez. We collected clinical, radiological and pathological data. We investigated the expression of mismatch repair (MMR) proteins in 214 patients and BRAF gene mutations in 159 patients.ResultsMean age was 55.08 +/− 15.16 years. 36.5 % of patients were less than 50 years old and 49.3 % of tumors were localized in the rectum. Loss of MMR protein expression was observed in 11.2 % of cases. It was independently associated with individual or family history of cancer belonging to Hereditary Non-Polyposis Colorectal Cancer (HNPCC) spectrum (p = 0.01) and proximal localization (p = 0.02). No BRAF mutation was detected in all cases.ConclusionsThese results confirm the high occurrence of CRCs to young patients and the high frequency of rectal localizations in Moroccan population. They mostly show an absence of BRAF mutation, supposing a rarity of MLH1 promoter hypermethylation pathway, which may even partially explain the CRC peculiarities in our context.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5868184711716884

Introduction: Signet ring cell carcinoma is a rare tumor that generally originates in the gastrointestinal tract. Signet-ring cell carcinoma of the ampulla of Vater is extremely uncommon and a very rare clinical entity, which is infrequently reported in medical literature and only 31 cases have been mentioned. Most tumors affecting Vater's Ampulla are adenocarcinomas and other histological variants are less frequent. It mainly occurs in elderly patients. Case Presentation: We report a case of signet ring cell carcinoma of Vater's Ampulla. The tumour had infiltrated the duodenal, but local lymph nodes were clear (T3N0M0). Duodenopancreatectomy with pylorus preservation is the treatment of choice. Conclusions: Etiology and survival are not well-defined in the literature due to the extreme rarity of this disease.

Le carcinome mucineux primitif cutané est une tumeur annexielle rare, développée à partir des glandes sudoripares. Il se localise en générale à l'extrémité céphalique, surtout la région périorbitaire. Nous rapportons deux observations d'un carcinome mucineux primitif de la face et de la région axillaire, chez deux patients âgés de 60 ans. L’étude histologique montrait une prolifération tumorale dermohypodermique, faite de cellules organisées en cordons, en amas et en massif cribriformes, au sein d'une substance mucoïde. En immunohistochimie les cellules tumorales exprimaient la cytokératine 7, l'EMA et les récepteurs hormonaux. L'actine musculaire lisse a bien marqué les cellules myoépithéliales au niveau de la composante in situ dans un seul cas. Le carcinome mucineux primitif cutané est difficile à différencier d'une métastase mammaire ou digestive. La mise en évidence de carcinome in situ ou de cellules myoépithéliale est en faveur de l'origine cutanée primitive. C'est une tumeur à croissance lente, avec des métastases exceptionnelles. Le traitement est chirurgical et le taux de récidive est très élevé. Et à travers ces deux observations, les auteurs mettent en relief les principaux aspects cliniques, histologiques, thérapeutiques de cette entité avec une revue de la littérature.

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Introduction: Primary embryonal rhabdomyosarcoma of prostate (ERMP) is an extremely rare pathological and clinical entity in adults. It's characterized by extensive loco-regional spread and a tendency to metastasize by blood stream and regional lymphatics. In adult patients, data available on the management of ERMP are limited and mostly derived from the pediatric clinical trials on rhabdomyosarcoma. Case Report: We report a case of group III ERMP approached by initial surgery followed by the administration of three courses of vincristine, doxorubicin and Cyclophosphamide regimen. The patient succumbed to disease complications three months after the original diagnosis. Conclusion: The most appropriate therapeutic approach for adults with ERMP remains questionable. Further studies are needed to improve the understanding of biological behavior of ERMP in adults and to define the best therapeutic strategy in locally advanced disease.

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