Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series.

INTRODUCTION: Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present. AIM: We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution. CASES PRESENTATION: The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation. CONCLUSION: Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.

Multiple cutaneous metastasis of synchronous urothelial carcinoma of the bladder and the renal pelvis: a case report.

INTRODUCTION: Cutaneous metastatic disease arising from urinary tract carcinoma is rare and associated with a poor prognosis. We report a case of metastatic disease occurring in a patient treated for synchronous urothelial tumor of the bladder and left renal pelvis. CASE PRESENTATION: A 61-year-old Caucasian man was treated for a synchronous urothelial tumor of the bladder and left renal pelvis. He had an en bloc radical cystectomy and left ureteronehprectomy associated with a cutaneous transileal urinary diversion and lymph node dissection. He was scheduled for chemotherapy but was lost to follow-up. He consulted 1 year later with growing skin tumors that were confirmed to be metastatic disease, and he was referred to the oncology department for palliative chemotherapy. CONCLUSION: Cutaneous metastatic disease is a rare entity with poor prognosis. The main treatment remains chemotherapy; however, single-site metastasis should be considered for metastasectomy.

Lipaemia: an overrated interference?

Reagent method sheets for analysis of common serum analytes often highlight the possibility of interference from lipaemia but the information given is often brief and may not be instrument-specific. Thus study assesses the degree of interference from lipaemia in a range of common serum analytes on the Bayer Opera (with a serum blank) using a commercial polymer, LipoClear, as a lipid-clearing agent. Serum samples (mean serum triglyceride 6.89 [range 0.58-28.4] mmol/L) are analysed for 14 common chemistry analytes and the results compared before and after treatment with LipoClear. Results showed no significant critical differences in analyte values before and after treatment, except for an expected fall in total protein, phosphate, cholesterol and triglyceride concentrations. Most of the common analytes in use on the Bayer Opera are not subject to interference from lipaemia; however, we recommend that where method sheets indicate interference from lipaemia then this should be quantified for the analyte in question.

[Isolated acute peripheral facial paralysis in children. Etiological and prognostic study]. / Paralysies faciales périphériques aiguës isolées de l'enfant. Etude étiologique et pronostique.

BACKGROUND: As facial nerve palsy is usually of sudden onset and of obscure etiology, a survey of large series may provide new information. PATIENTS AND METHODS: The files of 40 children suffering from acute facial nerve palsy seen in 3 pediatric and 1 otorhinolaryngologic (ORL) departments between 1980 and 1990 were analysed. Patients with congenital palsy and those with paralysis that appeared after injury or in the course of acute or chronic disease (otitis media, tumor) were excluded from the study. The children were 9 months-16 years old (mean: 7 year 1 month). There were 22 left and 18 right facial nerve palsies. The palsy was moderate in 17 children, severe in 15, and total in 5. Etiologic factors (viral serologic studies, CSF examination), ORL studies and electromyogram of the facial nerve were analyzed in the majority of cases. Corticosteroids were used in 30 patients. RESULTS: A viral etiology was suspected and/or confirmed in 13 children; abnormalities were seen in 6 of the patients who underwent lumbar puncture. A familial facial nerve palsy was seen in 1 case and kidney disease in 2 cases. 23 cases appeared to be idiopathic. A moderate permanent residual weakness was seen in only 4 children. The mean time for recovery was 32 days (4-140 days). Corticosteroids did not influence the incidence of complete recovery, but decreased the time for recovery (26 versus 37 days) only in the patients who were treated before the 3rd day of the disease. Patients with idiopathic palsy recovered 23 days before those with viral etiology. CONCLUSIONS: Idiopathic and post-viral facial nerve palsies seem to have an excellent prognosis. Corticosteroids seem to have no effect although there is some evidence of benefit when treatment is begun early.