Djordje Savic

Torsion of malignant ovarian tumors in childhood and adolescence.

AIM To investigate the frequency of torsion of malignant ovarian tumors in children and adolescents. METHODS We evaluated all patients treated for adnexal torsion in a tertiary care referral pediatric institution during the last 20 years. Presentation, tumor markers and pathology reports were evaluated. We reviewed the literature on torsion of malignant ovarian tumors. RESULTS Ninety-two girls (age 6 months to 19 years), 41 of them premenarchal, were surgically treated for adnexal torsion. Symptoms and signs that led to clinical investigation and subsequent surgery were not specific. Histological findings of torsioned masses showed 69 non-neoplasms and 23 tumors, including five malignant. Origin of the malignant disease included four germ cell tumors and one sex-cord stromal tumor. The morphology index score for malignant tumors was > or = 7 in all five patients. Tumor markers were elevated in 12 patients, including four of the patients with malignant tumors. Complete staging was performed in three adolescents with stage Ia, IIa and IIIa of disease. We found tumor origin for 11 previous reported patients with torsion of malignant ovarian tumor, including seven germ cell and four granulosa cell tumors. CONCLUSION Torsion of malignant ovarian tumors in pediatric and adolescent patients occurs very rarely, but it is nevertheless possible at any stage of disease. The most common torsioned malignant ovarian tumors were of germ cell origin, in both premenarchal and postmenarchal girls. A torsioned adnexal mass with index > or = 7 needs to be considered as a potential malignant tumor.

Pre-operative Differentiation of Pediatric Ovarian Tumors: Morphological Scoring System and Tumor Markers

OBJECTIVE To investigate the importance of morphological scoring systems in differentiation of ovarian tumors in childhood. METHODS Morphological assessment using DePriests index was performed for all patients with histopathological confirmation of ovarian tumor, with evaluation of tumor markers, from January 1997. RESULTS Fifty-three girls (age range 13 months to 19 years) were surgically treated for 59 ovarian tumors, including six bilateral. All lesions with cystic appearance on ultrasonography were benign, 23 of 35 semisolid, and four of ten solid tumors were also benign. Stage of malignant disease was as follows: stage I, ten; stage II, two; stage III, six. Sensitivity, positive predictive value and accuracy by DePriests and Uelands indexes for benign tumors (score <7) were: 0.88, 0.79; 0.89; and 0.94, 0.84; 0.93; respectively. Elevated levels of tumor markers were observed in 17 patients, including four patients with endocrine manifestations. In 24 patients ovaries were successfully preserved, including two patients with foci of immature teratoma in a dermoid cyst. CONCLUSION Ultrasonographic assessment with morphological analysis recommended by DePriest and Ueland is a very useful procedure for differentiating benign from malignant ovarian tumors in children. Tumor markers and endocrinological investigation are also useful for preoperative evaluation.

Downregulation of nicotinic and muscarinic receptor function in rats after subchronic exposure to diazinon

Highlights • We studied the development of tolerance to subchronic p.o. administration of DZN in rats, under both in vivo and in vitro conditions• As a consequence of AChE inhibition, ACh neurotransmitters are being accumulated in over stimulated nicotinic and muscarinic receptors.• With isolated diaphragm and ileum, we examined the down regulation of nicotinic and muscarinic receptor function through EFS technique.• The results of our research could be useful in forensic diagnostics of organophosphate poisoning.

Propionibacterium propionicus Infection in Chronic Granulomatous Disease

Correspondence Propionibacterium propionicus Infection in Chronic Granulomatous Disease Sir—Bourdeaut et al. [1] recently described 2 pediatric patients with chronic granulomatous disease (CGD) who developed chest infections caused by Pro-pionibacterium acnes. We describe a 7-year-old boy with CGD who developed a Propionibacterium propionicus infection. CGD is an inherited disease caused by defects in superoxyde-generating nicotin-amide adenine dinucleotide phosphate oxidase of phagocytes [2]. Impairment of oxygen-dependent intracellular killing mechanisms results in severe infections with catalase-producing Staphylococcus aureus, Burkholderia cepacia, or Aspergil-lus species [3]. In our patient, CGD was diagnosed at 3 months of age after an episode of sup-purative cervical lymphadenitis. X-linked inheritance of CGD was supported by the finding of intermediate values with the ni-troblue-tetrazolium (NBT) test (47% of neutrophils were NBT positive) of a blood sample from the patients mother. He was given prophylactic cotrimoxazole; he was free of major infections. At 7 years of age, he was admitted to our institute because of chest-wall contusion. At admission he was febrile (temperature, 39ЊC) and tach-ydispneic (respiration rate, 50 breaths/ min). Laboratory values were as follows: erythrocyte sedimentation rate, 84 mm/h; hemoglobin, 147 g/L; WBC count, 52.0 ϫ 10 9 cells/L, with 45.2 ϫ 10 9 neutro-phils/L; C-reactive protein, 237 mg/L (normal range, !10 mg/L). Chest radiography revealed a left-side pleural effu-sion. A thoracic CT scan showed microab-scesses of the left lower lobe. Chest drainage yielded 300 mL of fibrinous ex-udate. Empirical antibiotic treatment with tobramycin and ceftazidime was started. The patient became afebrile 48 h later but pleural effusion persisted, and a thoracot-omy was perfomed 2 weeks later. Pathological examination of the lung tissue specimen revealed microabscesses and granulomatous inflammation. Results of staining for mycobacteria and fungi were negative. Direct Gram staining of pleural fluid and lung biopsy specimens revealed non–acid-fast, gram-positive filamentous rods identified as P. propionicum. Isolates were sensitive to penicillin, cephalospo-rins, and vancomycin. We continued treatment with penicillin, and complete clinical remission was achieved 1 month later. The most common causes of pneu-monitis in patients with CGD are Aspergillus and Nocardia species and Burkholderia cepacia [3]. However, various opportunistic, catalase-producing microbes may infect patients with CGD [2, 3]. P. propionicus is a less common caus-ative agent of a disease similar to actino-mycosis [4]. Thoracic actinomycosis results from aspiration of pieces of infectious material from the teeth and may involve the chest wall, the lungs, or both. Intact mucosa are the first line of defense, because P. propionicus, …

Giant Gartner Duct Cyst and Elevated CA-125

BACKGROUND Gartner duct cysts represent vestigial remnants of the caudal end of the mesonephric (Wolffian) ducts. They are often associated with ureteral and renal abnormalities. In most cases they are solitary, less than 2 cm in diameter. Giant Gartner duct cysts are extremely rare. CASE We present a girl with a giant Gartner duct cyst, without notable associated abnormalities or pathology, successfully treated by surgical excision. Preoperative aspiration revealed elevated level of CA-125 in the cyst fluid, with normal serum level of the same marker. CONCLUSION Analyzing the aspirated fluid for CA-125 may be a useful tool for evaluation of cystic lesions in this region.

Appendiceal involvement in a patient with Gaucher disease

Almost any anatomical compartment may be involved in Gaucher disease (GD). Abdominal lymphadenopathy occurred during enzyme replacement therapy in more than a dozen children with GD so far. A fourteen-year-old boy from Serbia developed clinical signs of acute appendicitis six years after the onset of GD type 3 related abdominal lymphadenopathy. Ultrasound examination showed diffuse thickening of the intestinal wall in the ileocoecal region with periappendicular infiltration. An appendectomy was performed four months after conservative treatment with antibiotics. Histopathology revealed macrophages with cytological characteristics of Gaucher cells densely crammed in mesoappendiceal adipose tissue. Also the multifocal replacement of subserosal tissue by Gaucher cells and their infiltration to a variable depth of muscularis propria of the appendix were verified. Frank infiltration of the vermiform appendix with Gaucher cells represents a novel observation in a wide spectrum of manifestations reported in GD. A possible causative relationship of this infiltration with appendicitis is considered.

Extraskeletal Ewing sarcoma in the anterior abdominal wall

Online first: June 6, 2017 SUMMARY Introduction Extraskeletal Ewing sarcoma (ES) is a highly malignant neoplasm occurring most commonly in the thoracic wall and the paravertebral region. ES belongs to the group of small round cell tumors and displays pathognomonic structural abnormalities of the EWS gene. The aim of this article was to present extraskeletal ES in an extremely rare anatomic location, an unusual clinical presentation, and modified treatment strategy. Case outline A 15-year-old boy was admitted to the hospital with acute abdominal pain in the right iliac region. During urgent operation, because of suspected appendicitis with periappendicular infiltrate, partly hemorrhagic tumor tissue was discovered in the preperitoneal space. Histopathological and immunohistochemical analyses revealed a tumor resembling extraskeletal ES. A postoperative CT scan showed the tumor rest, which was completely removed in the second operation. Molecular genetic analysis confirmed extraskeletal ES by finding the EWSR1-FLI1 fusion gene. Chemotherapy and radiotherapy according to the VAC protocol were started, and the patient is free of the disease eight months after the first operation. Conclusion Our case is the fourth case of extraskeletal ES located in the abdominal wall, the second case confirmed by the molecular genetic finding, and the first case described in children at this anatomic site. Due to an extremely rare location, unusual clinical presentation, and needed genetic analysis, the tumor treatment strategy was modified with good short-term results.

Recurrent Pheochromocytomas in a Child with Familial von Hippel-Lindau Syndrome

To the Editor: von Hippel-Lindau (VHL) syndrome is a rare hereditary disease characterized by the presence of benign or malignant tumors, such are CNS hemangioblastoma, pheochromocytoma, retinal angioma, Bclear cell^ renal carcinoma, renal and pancreatic cysts [1]. VHL syndrome is inherited in autosomal dominant pattern with high penetrance, but 20 % of cases are new mutations. Pheochromocytomas have been described in 10–50 % of patients with VHL syndrome [2]. We present a case of 12-y-old boy with type 2 VHL syndrome who had successive development of bilateral adrenal pheochromocytomas, as well as extraadrenal pheochromocytomas. Symptoms were first noted at 5 y of age and included arterial hypertension of 161/110, fatigueness, sweating and headache. The diagnosis was established very quickly, as family data revealed that the boy’s father had VHL syndrome: pheochromocytomas, cerebellar hemangioblastoma and retinal angiomas. High levels of the adrenaline (52,36 μg/24 h), and noradrenaline excretion (153,11 μg/24 h) were detected in the urine, and ultrasound, CT scan and MIBG scintigraphy imaging discovered a tumor in the left adrenal gland. The proposed PET-CT was not available [3]. Genetic analysis of the patient showed presence of a germline mutation in exon 3 of the VHL gene in codon 167 (Arg 167Gln; CGG→ CAG), which was proved to be the same as his father’s. In the previous 7 y the boy had 5 operations, a total of Bde novo^ 7 adrenal and extraadrenal benign pheochromocytomas were removed, and bilateral adrenalectomy was performed. The patient received preoperative two-week alpha blocker phenoxibenzamine premedication. Some authors prefer prazosin and doxazosin as alpha blockers in preoperative premedication [4]. Cortical-sparing adrenalectomy was not feasible, but it is a dominant alternative approach for bilateral pheochromocytomas [5]. After operations the patient remained symptom-free, with normal urinary excretion of catecholamines and ultrasound, CT/MRI and MIBG exams. The recurrence of the pheochromocytoma used to be diagnosed on regular screening based on the above mentioned diagnostic procedures. Presently, the boy is feeling well, with life-long substutional therapy. The early manifestation of VHL syndrome in the index patient, frequent recurrences of pheochromocytomas Bde novo^ at new locations and the hereditary nature of the syndrome are the highlights of this letter. * Aleksandar Vlahovic aleksandarvlahovic@yahoo.com