Primary Endodermal Sinus Tumor of the Vulva: A Case Report and Review of the Literature

Background.Extragonadal endodermal sinus tumors arising in the external genitalia represent an exceedingly rare malignancy in women. Six cases of endodermal sinus tumors of the vulva have been reported to date, with three cases failing to respond to conservative surgery and vincristine-based chemotherapy. We report a seventh case of vulvar endodermal sinus tumor that was treated with radical surgery and platinum-based chemotherapy.Case.RT is an 18-year-old female who presented with a vulvar mass that was diagnosed as endodermal sinus tumor at the time of biopsy. She was subsequently treated with modified radical vulvectomy and ipsilateral groin lymphadenectomy, followed by bleomycin, etoposide, and cisplatin chemotherapeutic regimen. She has since remained free of disease for 18 months as evidenced by serum α-fetoprotein and physical exam at 18 months.Conclusions.Vulvar endodermal sinus tumors represent a very small number of germ cell tumors in women. Based on the previous accounts, this disease appears to be more fatal than endodermal sinus tumor arising at other sites. These tumors also have a predilection for local metastasis. Due to the previous accounts, we chose to treat this patient with radical surgery and platinum-based chemotherapy. This treatment regimen has resulted in a disease-free state for 18 months.     

Role of early EEG and neuroimaging in determination of prognosis in children with complex febrile seizure

BACKGROUND: The present study investigates the role of early use of EEG in children with no known neuropathology prior to the first CFS, and the contribution made by computed tomography (CT) and magnetic resonance imaging (MRI) to treatment and prognosis. METHODS: Over a period of 7 years, the authors evaluated 159 children (age range: 2 months-5 years) who were being treated for CFS at Haydarpasa Numune Training and Research Hospital, Pediatrics Clinic, Istanbul, Turkey, and who had no previously known neurological disorder. Patients who presented with febrile seizure were determined to have CFS if they fulfilled the following criteria: <3 months of age when seizure occurred, duration of seizure >/=15 min, more than one seizure occurred during a single episode of illness, or focal seizures and postictal neurological deficit was found. EEG was performed on all patients. CT was performed on the patients who had postictal neurologic deficit or focal seizures. Cranial MRI was performed on patients who had focal findings in their EEGs. RESULTS: Electroencephalogram abnormality was found in 71 cases; 51 of these were diagnosed with epilepsy during follow up. Six of the 16 cases whose EEGs were abnormal between days 2 and 6 were diagnosed with epilepsy. Twenty of the 30 cases whose EEGs were abnormal between days 7 and 10 were diagnosed with epilepsy. All 25 cases who had abnormal EEGs after day 11 were diagnosed with epilepsy. CT was performed for 36 patients, of which five were found to have pathological changes. Pathological changes were detected in two of the nine patients who had cranial MRI. Patients who received CT or MRI were all diagnosed with epilepsy during follow up. CONCLUSION: The results suggest that if neurological examination of CFS patients are normal after their clinical status has stabilised, EEG should be performed after 7 days at the earliest, however for the most accurate diagnosis EEG should be performed 10 days after CFS. The most important predictor for neuroimaging was found to be detection of postictal neurologic deficit. MRI had no advantages over CT in first treating CFS in the emergency unit.     

A new membrane-attack complex/perforin (MACPF) domain lethal toxin from the nematocyst venom of the Okinawan sea anemone Actineria villosa.

The Okinawan sea anemone Actineria villosa causes severe cases of stinging. We isolated the 60 kDa A. villosa toxin (AvTX-60A) as the major toxin from the isolated nematocysts of this species. AvTX-60A showed fatal toxicity to mice with intraperitoneal injection at a minimum lethal dose of less than 250 microg/kg. The N-terminal amino acid sequence was determined and the corresponding cDNA encoding AvTX-60A was sequenced. The deduced amino acid sequence of AvTX-60A showed high similarity with PsTX-60A, which had been isolated as one of the major toxins from the venomous sea anemone Phyllodiscus semoni. These sea anemone toxins are new members of the family of proteins containing membrane-attack complex/perforin (MACPF) domains, best known in pore forming proteins such as perforin. These are the first examples of MACPF domain proteins as toxins for prey acquisition or repelling predators in nature.