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Primary heart neoplasms are rare and poorly known by general pathologists. However, they are not exceptionally encountered in pathology laboratories having a recruitment of cardiac surgery specimens. About 80 % of them are benign tumors and 20 % are malignant tumors. Some tumors are specific to the heart or have characteristics when they grow in the heart, including myxoma and papillary fibroelastoma. The classification currently in use is the 4th edition of the WHO classification of heart tumors published in 2015, which takes into account the evolution of knowledge in the field of sarcomas, but which does not yet recognize cardiac intimal sarcoma. recently individualized in its intracardiac location., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

We report the case of a girl of 5 and a half months admitted for discomfort and consciousness loss at home and supported on sudden infant death protocol. Workup was negative. Autopsy showed only signs of asphyxia. Microscopic examination of the pancreas showed hypertrophic beta cells of Langerhans islets, explaining death linked to severe hypoglycemia by inappropriate insulin hypersecretion. This observation highlights the importance of the management of sudden infant unexpected death according to the protocol of the National Health Authority, which includes an autopsy with complete sampling, which in this case resulted in a diagnosis of unknown disease the lifetime of the child., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

A 59-year-old male, was admitted to our hospital for a tumor of the pancreatic tail. Serum CEA and CA 19-9 levels were normal. Splenopancreasectomy found a desmoid tumour. A 69-year-old male was referred to our institution for chronic anemia and inflammatory syndrome with splenomegaly. Splenectomy showed an important splenic congestion and siderosis. Both patients had a type 2 diabetes mellitus. Furthermore, histological examination revealed pancreatic endocrine microadenomas. The two patients' postoperative course was unremarkable. Eleven and 24 months respectively after the diagnosis, the patients are alive and well, with no tumor recurrence., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Lipomatous polyposis of the colon is a rare affection, with about 10 published cases. This affection is characterized by a great number of polyps which can reach several hundreds, of diffuse location on the entire length of the colon, with a higher density on the left colon and the sigmoid. The size of polyps varies from 1 to 10 cm. When they are voluminous, they can be cause of obstruction, ulceration or bleeding. We report a case of colonic lipomatous polyposis, associated with an ileocolic lipomatous, an ileocolic diverticulosis and adenomatous polyps in a 70-year-old man.

Hereditary non polyposis colorectal cancer or Lynch syndrome is due to germline mutation of one the DNA mismatch repair genes. This mutation is associated with an unstable phenotype in tumor DNA characterized by new microsatellite alleles that are absent in matching normal DNA. Besides the Bethesda reference panel, a new panel of 5 mononucleotide microsatellites (BAT25, BAT26, NR21, NR24, NR27) has been proposed, which is more sensitive and faster to use in a multiplex PCR assay. In tumor cells, immunohistochemistry detects the loss of expression of either MLH1, MSH2, MSH6 or PMS2 protein, corresponding to the mutated gene. Immunohistochemistry guides germline analysis, except for MLH1 extinction which may correspond to either MLH1 germline mutation or methylation of MLH1 promoter resulting in inactivation. The latter is mostly due to aging and is often associated to the V600E BRAF gene mutation in tumor DNA. Combination of these 3 somatic analyses should reduce indications of germline mutation analysis in Lynch syndrome.

Hirschsprung's disease (1/5000 live births) is defined by the congenital absence of neuronal cells in the nervous plexuses in the distal part of the digestive tract. The disease affects the rectum and sigmoid colon in 80% of cases, or is more extensive. Hirschsprung's disease is suspected in cases of low gastrointestinal obstruction in the neonatal period, or in cases of chronic severe constipation in childhood. It is diagnosed by pathological examination of rectal biopsies that include the submucosa. After standard staining, multiple sections are scrutinized for neuronal cells. Acetylcholinesterase staining is performed on a frozen fragment to demonstrate the hyperplasia of cholinergic fibers that is very suggestive of Hirschsprung's disease. This hyperplasia decreases from the rectum to the splenic flexure of the colon. Hyperplasia of extrinsic nerve fibers and rarefaction of neuromuscular junctions in Hirschsprung's disease may be demonstrated immunohistochemically. Differential diagnosis includes chronic intestinal pseudo-obstructions. The treatment for Hirschsprung's disease is, most often, anastomosis of the normally innervated gut to the anal canal. Peri- or pre-operative biopsies assist surgery, but their interpretation is difficult in the transitional zone. The examination of the surgical specimen allows measurement of the aganglionic segment and transitional zone. Different genes (RET, most often) may be involved in sporadic or familial Hirschsprung's disease. Hirschsprung's disease is associated with other digestive or extra-digestive abnormalities in 5 to 30% of patients. Associated abnormalities may delay the diagnosis and treatment of Hirschsprung's disease.

Pancreatic neuroendocrine tumors are rare in von Hippel-Lindau disease, most often asymptomatic, nonfunctioning, non secreting, and benign. We report a case of low grade malignant pancreatic, secreting and asymptomatic neuroendocrine tumors, occurring in a 27 year old woman in the setting von Hippel-Lindau disease with recurrent pheochromocytoma, retinal and medullary hemangioblastomas, paraganglioma of the carotid body and ovarian cystadenoma. Neuroendocrine pancreatic tumors of von Hippel-Lindau disease are often constituted by clear cells, in the contrary of other neuroendocrine tumors of the pancreas. Occurrence of a pancreatic neuroendocrine tumor, especially in association with pheochromocytoma, may be misdiagnosed with a type 2 multiple endocrine neoplasia syndrom instead of von Hippel-Lindau disease.

Cholesterol crystal embolism is a serious complication of atherosclerosis responsible for renal, cutaneous and less often digestive manifestations. We report a case of cholesterol crystal embolization in an adenomatous colonic polyp with foci of adenocarcinoma in a patient with an abdominal aortic aneurysm taking anticoagulant therapy. This case shows that cholesterol crystal embolization may be diagnosed by microscopic examination of a colonic polyp.

The histological study of gastric biopsies is very much used for the diagnosis of Helicobacter pylori infection. A survey was performed among the members of the "Club d'Histopathologie Digestive et Hépatique" on the conditions of this diagnosis. The diagnosis is performed systematically by 91% of the pathologists. A special stain is used in 72% of cases (most often Giemsa), and immunohistochemistry is very rarely used. The classifications of gastritis that are frequently used are the Sydney system and the Whitehead's classification. In conclusion, it appears that the methods in use in France for the histological diagnosis of Helicobacter pylori infection are relatively homogeneous.

Tuberous sclerosis is an autosomal dominant disease characterized by the development of multiple hamartomas which involve mainly the skin, the brain, the kidneys, and the heart. Vascular involvement is uncommon, with essentially several reports of aortic aneurysm. The present case concerns a two and a half year-old girl with known tuberous sclerosis, who underwent a graft replacement of an abdominal aortic aneurysm revealed by hypertension, associated with a right nephrectomy for multiple renal angiomyolipomas and cysts. Microscopic examination of the aortic aneurysm and the right renal artery showed fibromuscular dysplasia. This case and the rare previous reports emphasize the possible vascular involvement in tuberous sclerosis, and suggest that arterial fibromuscular dysplasia may represent an additional manifestation of the disease.

Whipple's disease and pulmonary and lymph node tuberculosis have been diagnosed in a 70 year-old man hospitalized for weight loss and fever. The tuberculous or Whipple's disease-related nature of associated non-necrotizing epithelioid hepatic granulomas could not be determined. The existence of cellular immune defects in Whipple's disease could explain the association with tuberculosis, which has not been previously reported in the literature.

Thyroid carcinomas with clear cell change are rare neoplasms, and always bring up the possibility of a metastasis from another primary clear cell carcinoma. We report the case of a 33-year-old woman operated on for a solitary cold thyroid nodule. Histologic and ultrastructural studies showed a clear cell carcinoma with a trabecular and follicular pattern. Neoplastic cells included lipid droplets, glycogen granules and intranuclear cytoplasmic inclusions, and were stained with one of the two antithyroglobulin polyclonal antibodies used. Radiologic investigations then showed a tumor mass of the right kidney. Surgical management proved it to be a benign cyst, and assessed the absence of kidney neoplasm. Thus, this case exemplifies the pitfalls in the diagnosis of clear cell thyroid neoplasms, and points out the fact that immunohistochemical staining for thyroglobulin should be systematically performed.