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Resume Le rhabdomyosarcome est une tumeur mesenchymateuse maligne a differenciation musculaire striee. Sa variante sclerosante est une entite rare, decrite dans la derniere edition de l’OMS des tissus mous, associee au sous-type a cellules fusiformes, avec lequel elle partage des caracteristiques cliniques, morphologiques et cytogenetiques. Les avancees de la cytogenetique ont permis une approche pronostique des rhabdomyosarcomes a cellules fusiformes/sclerosants en individualisant 3 groupes genomiques de pronostic different. La localisation parotidienne du rhabdomyosarcome sclerosant est exceptionnelle avec seulement deux cas rapportes dans la litterature. Elle pose des problemes de diagnostic differentiel avec d’autres tumeurs malignes essentiellement epitheliales. Nous rapportons le 3e cas de rhabdomyosarcome sclerosant de la parotide, diagnostique chez une fillette de 7 ans qui avait consulte pour une tumefaction indolore de la parotide droite evoluant depuis 4 mois. Sur la piece de parotidectomie partielle, la tumeur mesurait 35 × 30 mm, etait mal limitee, d’aspect blanchâtre et translucide a la coupe. Histologiquement, elle etait constituee de travees et de cordons de petites cellules rondes, munies de noyaux ovalaires souvent irreguliers avec de nombreuses figures de mitoses. Le stroma etait abondant et hyalinise. De rares cellules d’aspect rhabdomyoblastique etaient notees. Le diagnostic de rhabdomyosarcome sclerosant a ete retenu devant la positivite immunohistochimique de la desmine, de l’actine muscle lisse et de la myogenine. Il n’a pas ete realise d’etudes cytogenetiques ou moleculaires. La patiente a eu une radio-chimiotherapie adjuvante, sans signe de recidives ou de metastases apres un recul de 8 ans.

Cribriform-morular thyroid carcinoma (CM-TC) is a rare entity that usually occurs in association with familial adenomatous polyposis (FAP) but may be sporadic. Herein, we present a new case of cribriform-morular thyroid carcinoma occurring in a 28-year-old woman with no history of FAP.

Intestinal infestation with Enterobius vermicularis is common, especially in the developing countries. However, its migration in the bile ducts is rare, often diagnosed after cholecystectomy. More investigations are needed to define its involvement in symptoms presented by patients and the likelihood of complications.

Metatypical basal cell carcinoma (MBCC) represents a high-risk type of cutaneous tumor and has characteristics similar to basal cell carcinoma and squamous cell carcinoma. We report a retrospective study of 13 patients who presented with cervicofacial MBCC. Our study found an increased prevalence of aggressive clinicohistologic features and showed disease recurrences in more than one-third of the patients; therefore, we conclude that MBCC is an aggressive variant that requires a complex surgical approach for achieving a stable and complete remission. (

Introduction : Ameloblastoma is the most common aggressive benign odontogenic tumor of the jaws. This kind of tumor arises from dental embryonic remnants. Clinical case : We report a case of acanthomatous ameloblastoma of the mandible occurring in a 79-year-old male patient. Through this case, we describe clinical and radiological findings in ameloblastoma, discuss different variants of this tumor, and emphasize the histological diagnosis. Discussion : Among the various types of aggressive benign odontogenic tumors, acanthomatous ameloblastoma represents a subtype of the solid multicystic ameloblastoma with specific microscopic features. Its course is controversial. Conclusion : Although the diagnosis of acanthomatous ameloblastoma is based on histologic features, dentists and oral surgeons should consider this possibility in patients with an aggressive tumoral process.

HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.

HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD.

Rhabdomyosarcoma is a malignant mesenchymal tumour with skeletal muscle differentiation. Its sclerosing variant is a rare entity, which is described in the latest WHO edition of soft tissues in association with the spindle cell subtype, with which it shares clinical, morphological and cytogenetic features. Cytogenetic advances have allowed a prognostic approach to fusiform/sclerosing cell rhabdomyosarcoma by individualizing 3 different genomic prognostic groups. The parotid location of sclerosing rhabdomyosarcoma is exceptional with only two reported cases in the literature. It can pose a diagnostic challenge because of its rarity and histological similarities with other malignancies. We report the third case of sclerosing rhabdomyosarcoma of the parotid gland, which occurred in a 7-year old girl, who had consulted for a painless swelling of the parotid region of 4 months duration. Gross examination of the partial parotidectomy demonstrated an ill-circumscribed 35×30mm, firm and white glistening tumor. Histologically, it was composed of cords and trabeculae of small round cells, with ovoid and often irregular nuclei. Mitoses were numerous. The cytoplasm was scanty and cell margins were unclear. Tumor cells were set in a prominent hyalinized matrix. Scattered rhabdomyoblastic-like tumor cells were noted. The diagnosis of sclerosing rhabdomyosarcoma was performed after the positive immunostaining with desmin, myogenin and smooth muscle actin. No cytogenetic or molecular studies were performed. The patient underwent adjuvant chemo and radiotherapy, without recurrences or distant metastases during the 8-year follow-up.

Resume Les tumeurs vitellines (TV) sont des tumeurs germinales (TG) rares qui surviennent le plus souvent au niveau de l’ovaire et du testicule. Les localisations extragonadiques sont rares et sont surtout sacrococcygiennes, mediastinales, cerebrales, retroperitoneales ou encore vaginales. L’atteinte peritoneale primitive est exceptionnelle. Nous rapportons l’observation d’une jeune fille âgee de 16 ans operee en urgence d’une volumineuse masse peritoneale associee a des nodules hepatiques et compliquee d’un hemoperitoine ; une tumorectomie et une resection hepatique segmentaire ont ete realisees. A l’examen histologique, cette tumeur repondait a une TV. La patiente a eu une chimiotherapie intensive, elle va bien avec un recul de deux ans. A la lumiere de cette observation, nous nous proposons de discuter les particularites anatomocliniques de cette tumeur.

Endobronchial hamartochondroma is a form of rare benign tumour. Compared to those that occur in the lung parenchyma, the endobronchial form can potentially be managed by relatively conservative treatment involving per-endoscopic resection.A 61-year-old patient had a dry cough and chest pain for 3 months. Their clinical examination was normal, but thoracic CT scan showed lingular collapse. Bronchoscopy revealed the presence of a multilobar tumour occluding the orifice of the lingula bronchus. Bronchial biopsies were consistent with the diagnosis of a hamartochondroma. Before the destruction of any of the left upper lobe parenchyma, the tumor was resected surgically and the patient had an uneventful postoperative course.Endobronchial hamartochondroma support must be rapid to avoid irreversible parenchymal consequences downstream obstruction involving an often mutilating surgery. Essentially endoscopic treatment should be the most conservative possible.

Yolk sac tumours are rare germinal neoplasms that often arise in ovary and testis. Extragonadal localisations such as mediastinum, retroperitoneum, brain and vagina are uncommon. Primary intraperitoneal tumours are exceedingly rare. We report a case of a 16-year-old girl who underwent laparotomy for an acute abdominal pain and circulatory dysfunction. Abdominal exploration disclosed a large mass involving mesentery of the transverse colon associated to three hepatic nodules and abundant peritoneal bleeding. Segmental hepatic resection was performed and the mesenteric mass was removed. Microscopic examination of the pathologic specimen concluded to a yolk sac tumour. Patient underwent intensive chemotherapy; she's free of disease 2 years after diagnosis. Through this case, clinicopathologic features of this rare neoplasm will be discussed.

The study was designed in order to evaluate the degree of correlation of mitotic index (MI), Ki67 (MIB1) score and S-phase fraction (SPF) as markers of cell proliferation and prognosis in breast cancer.The series analysed corresponded to 257 consecutive invasive breast carcinoma, treated at the Institut Curie, France, in 1995. Nottingham histological grade and MIB1 semiquantitative and quantitative score were assessed on histological sections, whereas SPF was calculated using flow cytometry analysis of fine-needle aspiration products. Proliferation indices were compared to pathological data and to overall survival (OS) and disease-free survival (DFS) (minimum follow-up: 72 months).The median values for the proliferation markers were 9/10 HPF for MI, 32.4% for MIB1 and 3.7% for SPF. A high rate of correlation (r=0.96; p0.001) was observed between semi-quantitative and quantitative MIBI evaluation. A positive correlation was found between the three markers (r ranging from 0.54 to 0.61;p0.001). Univariate analysis of markers associated to disease outcome showed that MIB1, axillary node status (N) and progesterone receptor (PR) status were significantly associated with OS and that MIB1 and SPF were associated with DFS, together with node and hormone receptor status. In multivariate analysis, when proliferation markers were adjusted on the N and PR status, only MIB1 retained a prognostic value for OS (RR= 1.83) [1.00;3.35] and SPF for DFS (RR= 1.58) [1.02-2.44] (p=0.04).A good level of correlation was observed between the values of the three markers of tumour cell proliferation analysed. In this series of invasive breast cancers, MIB1 immunostaining was found to be a prognostic marker of both OS and DFS. The median (32.4%) was a valuable cut-off value for prognostic assessment. Semi-quantitative and quantitative evaluations provided very similar values. MIB1 can thus be considered as a reliable prognostic maker, usable in small size tissue specimens which are inappropriate for MI or SPF analysis. The impact of MIB1 compared to that of the other proliferative markers will be further assessed in a subgroup of T1N0M0 for which the prognostic assessment is of major interest.