Undifferentiated Small Round Cell Sarcoma of the Hypopharynx in a 7-Month-Old Patient.

Undifferentiated small round cell sarcoma is an extremely rare and highly aggressive tumor touching the soft tissues and bones. Here, we report the case of a 7-month-old girl who presented to our department with respiratory distress due to a hypopharyngeal mass causing airway obstruction. The patient underwent a tracheotomy and had a direct laryngoscopy. Histological examination confirmed the diagnosis of an undifferentiated small round cell sarcoma. The patient received chemotherapy and radiotherapy and underwent neck dissection. One year later, the patient was diagnosed with local recurrence as well as metastatic cervical nodes and liver and pulmonary metastases. The case is original by the tumor's location and the age of onset. To the best of our knowledge, this could be the first case in English and French literature reporting an undifferentiated small round cell sarcoma affecting the hypopharynx among infants. The purpose of this article is to report our case and discuss its clinical and anatomopathological features as well as its prognosis and treatment options.

Evaluation of concordance of Gleason score between prostate biopsy and radical prostatectomy.

BACKGROUND: Histological Gleason score grading is a main determinant of prostate cancer treatment. However, the final histological examination may reveal that concordance rates between biopsy and pathological Gleason sums are inadequate. AIM: To evaluate the concordance of Gleason score between prostate biopsy and radical prostatectomy specimen and to study factors predictive of up-grading of Gleason score at radical prostatectomy. METHODS: We conducted a descriptive and retrospective study including cases of patients who underwent  prostatectomy between 2008 and 2015. We proceeded to a histological examination of 30 cases of radical prostatectomy and 17 corresponding biopsies. The data of the remaining 13 prostate biopsies, not performed in our hospital, have been picked from detailed histological reports. RESULTS: Our results showed that the concordance in the Gleason score was 43% (kappa = 0.11, poor agreement).Gleason score was upgraded in 54% of the cases. At radical prostatectomy, it increased by two  points in one case and by one point in 14 cases. The Gleason score was under-graded on prostatic biopsies in an only 1 case. Using the new classification ISUP 2014, the concordance rate was 26% (kappa = 0.04,  very poor agreement). Gleason score was upgraded in 78% of the cases for Group 1 (SG 3 + 3) and 63% for group 2 (SG 3 + 4). The concordance rate was highest for Group 4 (4 + 3). Variables as age, serum PSA (prostate specific antigen) , numbre of cores, percentage of positive cores, or prostate volume were not significant predictors of upgrading of Gleason score on radical prostatectomy specimen. CONCLUSION: Thus, the high rate of discordance of Gleason score between prostate biopsy and radical prostatectomy specimen implies an understanding of factors predictive of discordance of this score allowing urologists, pathologists and oncologists to support patients in a more suitable way, choosing the appropriate therapeutic modality for each patient.

Paratesticular Angiomyofibroblastoma-Like Tumor: Unusual Case of a Solidocystic Form.

The angiomyofibroblastoma-like tumor of the male genital tract is a rare benign tumor. A total of 34 cases have been reported in the literature. We herein report an exceptional case of solidocystic form in its paratesticular location, in a 79-year-old man. Clinical examination objectified a right testicular induration. Macroscopic examination of the orchidectomy objectified a paratesticular solidocystic tumor formation. Microscopically, the solid area was composed of vessels with small caliber and turgidity of endothelial cells. These vessels were surrounded by clusters of epithelioid cells, sometimes having the appearance of giant cells. They were associated with spindle cells. The cystic area was uncoated. Immunohistochemically, the fusiform cells expressed Desmin and Smooth Muscle Actin. Endothelial cells and clusters of cells surrounding large vessels expressed CD34. The expression of receptors for estrogen and progesterone was negative. To our knowledge, this is the second solidocystic case of angiomyofibroblastoma-like tumor of male genital tract.

Clinico-pathological and prognostic findings on 27 cases of medullary thyroid carcinoma.

INTRODUCTION: Medullary thyroid carcinoma is a rare tumor accounting for less than 10% of thyroid neoplasm. This tumor is characterized by important histological polymorphism which makes morphological diagnosis difficult and immunohistochemical study often necessary. OBJECTIVE: We aim to perform a retrospective review of clinical and pathological characteristics of medullary carcinoma. We will discuss the place of immunohistochemistry in the positive diagnosis and as a prognostic factor. METHODS: patients with thyroid medullary carcinoma diagnosed in department of pathology at carcinologic institute between 1998 and 2013 were retrospectively included. Clinic, radiologic and prognostic variables were assessed. Slides were reviewed for all the patients with confirmed tumors. RESULTS: Twenty-seven patients with CMT were identified. The average age was 55 years with predominance of males. The average consultation time was 16 months. The most common presentation symptom was a cervical lymph node. Total thyroidectomy was performed in 23 patients. Tumor was nodular and unique in 22 cases. The average size was 2.1 cm. CMT was of mixed type containing both medullary and papillary compound in four cases. Amyloid substance was present and abundant in 21cases. Positive staining for calcitonin was observed in 16 cases. Distant metastasis or metastatic lymph nodes was observed in eight cases with an average period of 42 months. Radiotherapy was performed in fifteen cases and two patients received chemotherapy. CONCLUSION: In the absence of amyloid deposits, immunohistochemical staining with calcitonin is useful to confirm the diagnosis. The prognosis of this entity is more pejorative than papillary thyroid carcinoma.

[Prognostic value of markers of aggressiveness of urinary bladder tumours evaluated by immunohistochemistry]. / Valeur pronostique des marqueurs d'agressivité des tumeurs vésicales évalués par immunohistochimie.

BACKGROUND: The study of some immunohistochemical markers provides an objective and reproducible prognostic evaluation of urinary bladder tumour. aims: study the expression of the following immunohistochemical markers in Tumours of the bladder: Proliferating Cell Nuclear Antigen (PCNA), Ki67 antigen (MIB1), the C-erbB2 proto- antigene, the tumor suppressor gene p53, the receptor for epidermal growth factor (EGF-R), the apoptosis suppressor gene bcl2, the carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). methods: Study of retrospective series of 30 patients having tumours of the urinary bladder. results: The expression of PCNA with a cut-off value of 14% is correlated with recurrence (P = 0.010). The expression of PCNA with a cut-off value of 1% is correlated with tumour stage (P = 0.003). The expression of MIB1 with a cut-off value of 47% is correlated with recurrence (P = 0.010). The expression of MIB1 with a cut-off value of 47% is correlated with the tumour progression in stage and\or in grade (P = 0.007). The expression of C-erbB2 with a cutoff value of 28% is correlated in the tumour grade (P = 0.007). The other antibodies didn't demonstrate a prognostic value. CONCLUSION: MIb1 and PCNA being correlated with recurrence, they can be useful with the decision of the rhythm of the endoscopy. The correlation of C-erbB2 with the tumour grade could serve to better graduating bladder tumours.

[Solitary fibrous tumor of the pancreas. A case report]. / Tumeur fibreuse solitaire du pancréas. A propos d'un cas.

Solitary fibrous tumour (SFT), a rare mesenchymal neoplasm usually arising from the pleura, may also occur in many other extra pleural sites. It has exceptionally been described in the pancreas. This report describes the case of a benign SFT of the pancreas occurring in a 41-year-old man who presented with a solid epigastric mass. Pathological and immunohistochemical findings are presented. Imaging features on ultrasonography, CT, MRI, and arteriography are widely detailed. Surgical resection of the tumour was performed, and the patient died from postoperative complications.

[Non-Hodgkin's malignant lymphoma: a rare cause of testicular tumour]. / Le lymphome malin non hodgkinien: une cause rare de tumeur testiculaire.

The authors report a clinicopathological case of non-Hodgkin's malignant lymphoma of the testis in a 66-year-old man. The diagnosis was established on a right orchidectomy specimen. Conventional histopathological examination and immunohistochemistry concluded on diffuse large B-cell lymphoma. Staging showed involvement of Waldeyer's ring and the presence of retroperitoneal and para-aortic deep lymphadenopathy.

[Epithelioid hemangioma of bone: a rare pathologic entity]. / Hémangiome épithélioïde primitif de l'os: une entité rare.

Epithelioid hemangioma of bone is a rare benign vascular lesion. Since its first description by Rosai in 1979 about thirty cases have been reported. We report two new cases diagnosed in patients aged 21 and 7 years. Both patients had multiple lesions on the legs. Bone pain was the main symptom. Routine laboratories studies were unremarkable. Bon radiographs showed an expansive process of the bone. Histological and immunochemical features were typical of epithelioid hemangioma of the bone. Treatment consisted in above knee amputation for the first patient and therapeutic abstention for the second. Both patients are alive without progressive local disease or metastasis.