Clinical predictive factors of histological chorioamnionitis: case-control study.

INTRODUCTION: Histological chorioamnionitis or "intrauterine inflammation or infection" (Triple I) it is an acute inflammation of amniotic membrane, chorionic plate and umbilical cord. SUBJECT: To assess in the event of the clinical predictive factors associated to histological chorioamnionitis. METHODS: Prospective examination of 50 placentas from aberrant pregnancies, and 50 placentas from 'normal' deliveries. The Placentas analyzed by the conventional histopathology method, and the severity of chorioamnionitis was classified histologically according to the intensity and the topography of placental inflammation.The clinical and histopathological features of the study groups were introduced into the SPSS 13 database (License University Mohammed V-Rabat). RESULTS: 36/50 placentas of aberrant pregnancies showed a histological chorioamnionitis often associated to a funisitis, and 11/50 normal placentas have shown some lesions of histological chorioamnionitis mainly grade one without funisitis.On the other hand we noted a statistically significant association between histological chorioamnionitis and premature rupture of the membranes (PROM) over than 12h (p < 0.001). CONCLUSIONS: Our study confirmed the predominance of histological chorioamnionitis lesions in clinically suspected cases of chorioamnionitis with 72% versus 22% in the controls group.Among the clinical parameters studied, only the premature rupture of the Membranes was shown a statistically significant association with the appearance of histological signs of chorioamnionitis.In conclusion, chorioamnionitis is sometimes clinically silent. Morphological placental study could be a confirmation of this pathology, which is predominantly associated to PROM over than 12 h.

C3 glomerulopathy associated to multiple myeloma successfully treated by autologous stem cell transplant.

A 32-year-old male presented with advanced renal failure and nephrotic proteinuria due to lambda light chain multiple myeloma. Renal biopsy showed a proliferative glomerulonephritis with isolated C3 deposits. Renal recovery was obtained after chemotherapy and autologous stem cell transplant. We review previously described cases of C3 glomerulopathy associated with monoclonal gammopathy.

Kinetics of parathyroid hormone after parathyroidectomy in chronic hemodialysis patients.

Secondary hyperparathyroidism is a common complication in chronic renal failure. The treatment in some cases requires parathyroidectomy. The kinetics of the parathyroid hormone (PTH) levels after surgery helps to evaluate the efficacy of parathyroidectomy. Prospective analysis was made of the kinetics of intact PTH (iPTH) after parathyroidectomy in 10 chronic hemodialysis (HD) patients who had secondary hyperparathyroidism. We determined the levels of iPTH before surgery and its evolution after parathyroidectomy at regular intervals: Day 0, D7, D15, D30 and D90. The mean age of our patients was 40 ± 13 years, with a sex ratio of 1. The mean duration on HD was 122 ± 63 months. The duration of secondary hyperparathyroidism varied from one year to 12 years. All patients had received medical treatment for hyperparathyroidism. The indications for parathyroidectomy included resistance to medical treatment in seven cases, development of brown tumors in two cases and soft tissue calcifications in one case. All patients had radiographic evidence of hyperparathyroidism. The parathyroidectomy was sub-total in all patients, 6/8 in four cases and 7/8 in six cases. The mean iPTH level was 2341 ± 1946 pg/mL before surgery. A sharp drop in this level was noticed on D0, with a median of 92 pg/mL and, thereafter, the levels were 79 pg/mL on D7, 25 pg/mL on D15 and 36 pg/mL after 1 month. At 3 months post-surgery, the mean iPTH level was 302 pg/mL. Histological examination of the resected gland showed parathyroid hyperplasia in all patients. In our series, the efficacy of sub-total parathyroidectomy was satisfactory with rapid normalization of PTH, which is consistent with the literature data. Sub-total parathyroidectomy still has a place in the treatment of secondary hyperparathyroidism in chronic renal failure. Its indications should be limited to cases resistant to medical treatment and, in particular, in cases with occurrence of complications.

[Renal amyloidosis revealing a Kartagener's syndrome]. / Amylose rénale révélant un syndrome de Kartagener.

Kartagener's syndrome is a rare disease. It is a clinical variant of primary ciliary dyskinesia. It combines a situs inversus, chronic sinusitis and bronchiectasis. Glomerulopathies associated with this syndrome are rarely reported. We report the case of Kartagener's syndrome in a patient diagnosed at the age of 37 years with renal amyloidosis.

[Botryoid Wilms tumor extending into the duodenum]. / Néphroblastome botryoïde étendu au duodénum.

We report on a rare case of botryoid Wilms tumor extending into the duodenum. This uncommon macroscopic form of nephroblastoma consists entirely of a polypoid renal intrapelvic mass. The main differential diagnosis of this unusual tumor is botryoid rhabdomyosarcoma. A 14-month-old boy presented with a painful abdominal mass. Radiology revealed a large heterogeneous mass in the renal calyx, protruding into the ureter. A right radical nephroureterectomy was carried out. The tumor was found to extend into the 2nd portion of the duodenum. The pathologic diagnosis was mixed type nephroblastoma, SIOP 2001 stage III. The patient was given a course of postoperative chemotherapy. No local recurrence or metastatic spread has been detected after 2 years. Only few such cases have been previously reported, some of them extending into the bladder. To our knowledge, botryoid nephroblastoma has not previously been described as extending into the digestive system.

[Clear cell sarcoma of soft tissues: a case report]. / Le sarcome à cellules claires des parties molles à propos d'un cas.

Clear cell sarcoma or melanoma of soft tissues is a rare tumor in young adults, mainly located in the extremities. We report a new case observed in a 15-year-old teenager who had injured her left knee 1 month before hospitalization. The patient consulted for deformation of the left knee with pain and limitation in flexion. A CT scan of the knee showed a hypodense mass of the anterior compartment of the left leg. The histological and immunohistochemical study of the biopsy fragment concluded in clear cell sarcoma of the soft tissue. The histogenesis of this tumor continues to be debated and raises diagnostic challenges for the pathologist who must eliminate melanoma metastases, whose prognosis is different.

[Glomerulonephritis and thymoma: a case report and review of the literature]. / Glomérulonéphrite et thymome : un cas clinique et revue de la littérature.

The authors present a case of nephrotic syndrome due to focal-segmental glomerulosclerosis, occurring three years after thymectomy and myasthenia gravis. Twenty-three other cases of nephrotic syndrome associated with thymoma and myasthenia gravis have been reported in the literature. The nephrotic syndrome may be related to T-cell dysfunction associated with thymoma.

Myocardial lysis in a fetus induced by maternal paraphenylenediamine poisoning following an intentional ingestion to induce abortion.

The acute toxicity of paraphenylenediamine (PPD) has been associated with several histopathological changes. In humans, acute PPD poisoning is known to cause rhabdomyolisis and particularly myocardial lysis. However, its toxicity for the fetus has never been reported in the literature. We report a case of myocardial lysis in a fetus expelled by a 22-year-old mother after apparent ingestion of an unknown amount of PPD. The patient was admitted to our intensive care unit with acute onset of respiratory distress and rhabdomyolysis. The pelvic ultrasonography on admission showed a normally progressing pregnancy of 23-24 weeks. On day 9 post-ingestion, the patient spontaneously expelled a non-viable fetus. The fetal examination did not show any external or macroscopic abnormalities. However, the histopathological exam showed an important heart and lung congestion. There was also some interstitial edema and inflammation at the base of the lingua, in addition to a chorionic villus thrombosis and abruptio placentae. The histopathology of the myocardium showed lysis of the cardiac muscle. This observation suggests that the PPD was most likely responsible for the myocardial injury in the fetus.

[Clear cell sarcoma of the kidney. A study of 13 cases]. / Sarcome rénal à cellules claires. A propos d'une série de 13 cas.

UNLABELLED: Clear cell sarcoma of the kidney (CCSK) also called a "bone-metastasizing renal tumor of childhood" is the second common pediatric renal neoplasm. This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms' tumor. PATIENTS AND METHODS: We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children's hospital of Rabat between 1990 and 2002. RESULTS: The median age at diagnosis was 14 months (5 months-9 years). The male to female ratio was 5.5:1.00. Abdominal mass, usually the first physical finding, was associated with hematuria in four cases. No congenital malformation syndrome or familial Wilms' tumor were observed. Imaging studies found out seven right and six left intrarenal processes. Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols. Twelve of 13 children underwent nephrectomy. Tumor measurements varied through 450-3450 g and 7-26 cm. The classic morphologic pattern was seen in nine cases (69%). The distribution local stage was I: three cases; II: three cases; III: six cases; IV: one case. Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases. With a median follow up of 44 months, four patients showed bone metastases (31%), four are alive in CR, four are lost for follow up and five died. CONCLUSION: CCSK remains the pediatric renal tumor most frequently misdiagnosed. Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.

[Chondromyxoid fibroma of bone: a rare benign bone tumor in children]. / Le fibrome chondromyxoïde de l'os: une tumeur osseuse rare de l'enfant.

Chondromyxoid fibroma is a rare benign tumor that is typically found in the metaphyseal ends of long tubular bones, such as the tibia. The radiographic appearances are those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. The classic histological feature of a chondromyxoid fibroma is stellate or spindle-shaped cells arranged in lobules in a myxoid or chondroid background. Two cases are presented here: 8, and 12-year-old patients, both with lesions in the proximal tibia. The first case showed an unusual feature: it was diaphyseal chondromyxoid fibroma. In the second case, the lesion was metaphyso-diaphyseal. The differential diagnosis includes chondroblastoma, myxoma, aneurysmal cyst as well as chondrosarcoma. A surgical conservative treatment with complete excision is recommended even in case of recurrence.

[Pleuropulmonary blastoma: possible interest of neoadjuvant chemotherapy. A case report]. / Blastome pleuropulmonaire: intérêt possible d'une chimiothérapie néoadjuvante. A propos d'un cas.

Pleuropulmonary blastoma is a very exceptional anatomoclinical and histological entity recently distinguished from adult pneumoblastoma. This tumor, observed in children aged less than 15, can involve the lung, the pleura, or the mediastinum and is characterized by a very poor prognosis. We report the case of a 4-year-old girl who developed pleuropulmonary blastoma which was discovered in a context of respiratory distress. Standard chest x-ray revealed an opacity covering the entire left lung. Histology and immunohistology led to the diagnosis of pleuropulmonary blastoma with several components: blastematous, malignant mesenchymatous with pluridirectional differentiation, and benign epithelial tissue. Treatment consisted in preoperative chemotherapy and radiotherapy to reduce tumor volume. This neoadjuvant treatment is not widely reported and its relatively favorable result allowed tumor resection. This approach might be useful in similar cases.

[Fibrocartilaginous mesenchymoma of bone. A case report]. / Mésenchymome fibrocartilagineux intraosseux. A propos d'un cas.

Fibrocartilaginous mesenchymoma of bone is a tumoral entity which is somewhat controversial. It has been delineated in 1984 by Dahlin and al. Fifteen cases have been reported in the literature. We report a new case which involves proximal humerus. X-ray data, microscopic findings and local recurrence indicate a low grade malignancy. Metastasis have never been reported. The main differential diagnosis are desmoplastic fibroma, fibrous dysplasia and fibrosarcoma with low grade malignancy. Treatment is surgical.

[Pleuro-pulmonary blastoma. Report of 4 cases]. / Le blastome pleuro-pulmonaire. A propos de quatre observations.

Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in children. Outcome is often unfavorable. Two boys and two girls, mean age 4.5 years, were admitted for nonspecific respiratory signs. Oriented by radiology findings, the diagnosis of pleuropulmonary blastoma was confirmed at pathology examination of a pneumonectomy specimen. Three of the children were given postoperative adjuvant chemotherapy. There were three deaths and one child was lost to follow-up. We discuss the clinical features of pleuropulmonary blastoma. No optimal treatment has been defined for this often fatal tumor.

Gene alterations and apoptosis in rhabdomyosarcoma.

The aim of the study is to look retrospectively for gene alterations and evaluate apoptosis in rhabdomyosarcomas (RMSs) from 40 children including 24 patients not previously treated. Histological subtype was botryoid in 1 case, spindle cell in 2 cases, embryonal in 22 cases, alveolar in 10 cases, and undetermined in 5 cases. Gene expression was evaluated immunohistochemically for p53 tumor suppressor gene, MDM2 oncogene, and bcl-2 gene. N-myc amplification was detected by in situ hybridization. Apoptotic cells and bodies were recognized morphologically and stained by 3-OH end labeling. Intranuclear accumulation of p53 protein was obvious (> 25% of tumor cells) in two recurrent embryonal RMSs. Expression of the MDM2 gene was intense (80% of tumor cells) in a recurrent and metastatic embryonal RMS. Amplification of the N-myc gene was obvious (about 20% of tumor cells) in an alveolar RMS metastatic at diagnosis. Expression of the bcl-2 gene was intermediate (25-75% of tumor cells) in 26% of cases and high (> 75% of tumor cells) in 10% of cases either embryonal or alveolar. The percentage of tumor cells showing morphologically recognizable apoptosis was 0.2-7.5% (mean 2.9%). There was no correlation between apoptosis and histological subtype, bcl-2 expression, or previous treatment.

[Localized pleural fibrous mesothelioma (submesothelial pleural fibroma). Anatomo-clinical study of 25 cases]. / Le mésothéliome fibreux pleural localisé (fibrome pleural sous-mésothélial). Etude anatomo-clinique de 25 cas.

We studied a retrospective series of 25 sub-pleural fibromas (benign localized pleural mesotheliomas) of which 23 where excised, collected over a period of 20 years. The current series confirms the excellent prognostic of these rare tumors. They are sometimes bulky, but not infiltrating and discovered by systematic chest X-ray. Modern imaging using CT-Scan and nuclear magnetic resonance can suggest their diagnostic which is confirmed by histopathologic analysis of the usually pediculate and easily removed tumor. Transthoracic needle aspiration is generally of little help for diagnosis. The histopathologic diversity of the tumor is rather confusing but characteristic features can be found with fusiform cells ans collagen stroma. Immuno-histochemistry is more helpful for diagnosis and histogenesis recognition than electron microscopy.