A case of Langerhans cell histiocytosis mimicking child abuse.

Langerhans cell histiocytosis is a rare non-malignant disease with clinical heterogeneity. The disease may present with various clinical findings and may imitate many other conditions. In this report we describe a 34-month-old girl who presented with chronic otitis and otorrhea, skull fracture, rash, vulvar edema, erythema and erosion in labia majors which initially suggested child abuse but the patient was diagnosed with Langerhans cell histiocytosis.

A girl with steroid cell ovarian tumor misdiagnosed as non-classical congenital adrenal hyperplasia.

Ovarian steroid cell tumors are rarely encountered in prepubertal girls. The majority of these tumors produce hormones, testosterone being the leading one. These tumors may either coexist with or imitate congenital adrenal hyperplasia (CAH). We present a 13-year-old female patient who was diagnosed with non-classical CAH at six years of age while being investigated for premature pubarche. She was diagnosed with steroid cell ovarian tumor after a delay of six years. The diagnosis was based on radiologic imaging, which was performed to investigate causes of unsuccessful metabolic control while under high-dose steroid therapy. The right ovarian hypoechoic mass of 23x22 mm was excised laparoscopically, preserving the ovary. Immunohistochemical staining showed that tumor cells were strongly positive with inhibin and focally positive with vimentin. Based on these findings, the patient was diagnosed with ovarian steroid cell tumor not otherwise specified. In the postoperative second week, total testosterone level was <10 ng/ml, and 17 hydroxyprogesterone (17-OHP) level was 1.1 ng/ml. Peak 17-OHP level was 4.2 ng/ml on repeated ACTH stimulations, and the diagnosis of CAH was excluded. Steroid therapy was tapered down and then discontinued. It should be kept in mind that there may be a misdiagnosis in cases of CAH, which may present itself with unsuccessful metabolic control even while under the appropriate treatment dose. Early diagnosis and treatment would prevent the development of irreversible signs.

Solid tumors in Turkish children: a multicenter study.

BACKGROUND: This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions. METHODS: The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out. Excluding epithelial tumors of the skin, leukemia, lymphoreticular system neoplasias, metastatic tumors, and hamartomas, 1362 solid tumors in 1358 patients were classified according to age, sex, localization, histopathology and clinical behavior. RESULTS: The male/female ratio was 0.9; 14.8% (201) of the patients belonged to 0-1 year age group, 20.7% (281) to 2-4 years, 25.9% (352) to 5-10 years, 22.2% (301) to 11-14 years, and 16.4% (223) to 15-18 years. Among all tumors, 708 (52.0%) were malignant, 645 (47.4%) benign tumors, 2 (0.1%) borderline tumors, and 2 (0.1%) unknown behavioral tumors. Malignant tumors were found in 50.2% (357) of female patients and in 54.0% (349) of male patients. A balanced distribution between benign and malignant entities among children under 18 years was observed. Comparison between the age groups revealed malignant cases outnumbered benign cases under 4 years of age while benign tumor numbers increased after 10 years of age. The most common entities in the malignant group were of sympathetic nervous system origin, while soft tissue tumors far outnumbered the others in the benign group. CONCLUSIONS: We conclude that the cancer patterns of children in the Ankara region mostly resemble with those of the western population. This study provides useful information on the diagnosis of solid tumors in children and highlights variations in cancer incidence in different age groups.

Invasive Aspergillus infection localized to the gastric wall: report of a case.

Invasive aspergillosis is most commonly seen in patients with immune disorders and usually in the lung. Local invasive aspergillosis of the gastrointestinal system is quite rare. A 13-year-old female without immune deficiency presented with acute abdomen due to full-thickness necrosis of the gastric fundus. The necrotic gastric wall was excised and the stomach repaired. The pathology revealed a gastric ulcer with invading Aspergillus hyphae and spores. Aspergillosis is an opportunistic infection and its spores cannot survive in the normal gastric mucosa. The Aspergillus spores in this case probably grew on a background of gastric ulcer and caused wall necrosis and that the surgical treatment possibly provided a cure because it remained localized to the gastric wall.

Foreign Body Reaction with High Standard Uptake Value Level in 18-FDG PET/CT Mimicking Relapse in an 8-Year-Old Patient Diagnosed with Hodgkin Lymphoma: A Case Report.

Combined positron emission tomography and computed tomography (PET/CT) using 18-fluorodeoxyglucose (18-FDG) is one of the most effective methods to identify pathological lymph node involvement. We report the case of a child who underwent cervical lymph node biopsy and chemotherapy for Hodgkin disease. Three years after surgery, PET/CT identified an intense localization of 18-FDG in the inferior cervical region. This finding led to a reexcision of the mass. The only finding was a granuloma arising in reaction to a hemostatic sponge.

Childhood fibroblastic and myofibroblastic tumors: a multicenter documentation and review of the literature.

OBJECTIVE: In this study, we aimed to give a documentation of 37 cases of childhood fibroblastic/myofibroblastic tumors retrieved from the archives of 6 reference centers in Ankara along with a comprehensive review on the subject. MATERIAL AND METHOD: A retrospective archive search was carried out for the period between 2006-2010 in 6 reference centers in Ankara covering patients with ages ranging between 0-18 years. All the tumors categorized under fibroblastic and myofibroblastic group according to World Health Organization criteria were collected. RESULTS: The study comprised 407 soft tissue tumors in total. Fibroblastic/myofibroblastic tumors constituted 9,1 % (37 cases) of these tumors. According to histopathology; 16 cases were categorized as fibromatosis, 8 cases as inflammatory myofibroblastic tumor, 6 cases as infantile fibrous hamartoma, 3 cases as nodular fasciitis and 2 cases as infantile myofibroblastic tumor/myofibromatosis and 1 case as cranial fasciitis. The only malignant case was an infantile fibrosarcoma. CONCLUSION: Infantile fibrosarcoma was lower than reported series and a male predominance was noted. The low incidence of newly described entities as well suggests that these tumors may have been unrecognized.

Incontinentia pigmenti with encephalocele in a neonate: a rare association.

Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis affecting ectodermal and mesodermal tissues. After the skin, the central nervous system is the second-most affected system. We report a neonate with incontinentia pigmenti and encephalocele, as a feature of the central nervous system involvement, to stress this uncommon association.

A huge ovarian mucinous cystadenoma in a 14-year-old premenarchal girl: review on ovarian mucinous tumor in premenarchal girls.

Superficial epithelial ovarian tumors are unusual in adolescent girls (when compared with adult women) and extremely rare before menarche. Mucinous cystadenoma (MCA) in children that is a rare form of epithelial tumor is a benign cystic ovarian neoplasm. To our knowledge, there are only eight cases of mucinous cystadenoma, three of borderline mucinous cystadenoma, and three of mucinous cystadenocarcinoma reported in the English-language literature. We present a 14-year-old premenarchal girl with a giant ovarian mucinous cystadenoma. This review is supported by the finding that epithelial ovarian neoplasms are extremely rare prior to puberty and that only 14 mucinous tumors have been reported prior to menarche.

Is Nothingham Prognostic Index correlated with apoptosis and p53 expression in infiltrating ductal carcinoma of the breast?

The role of p53 as a prognostic factor is not clear. P53 named as "guardian of the genome" plays an important role in many intracellular regulatory systems, one of which is apoptosis, having an impact on tumor kinetics. A retrospective study was undertaken to assess the relationship of the Nothingham Prognostic Index (NPI) to p53 expression and apoptotic cell counts. To conduct the study, 160 successive cases of infiltrating ductal carcinoma of the breast were included. P53 was assessed on AP-AAP stained sections. Apoptotic cell counting (ACC) was done on the HE stained routine sections in 10 HPFs. Clinical data were derived from the hospital files. Apoptotic cell counts were higher in the p53 positive group but the difference was not significant (p=0.079). P53 positivity was found to be related to the disease-free survival (DFS) (p=0.008). NPI was significantly higher in apoptotic cell containing group (p=0.006). There was a positive linear correlation between ACC and NPI scores (p=0.004). This correlation was not present between apoptosis and disease free survival. P53 expression was found to be related with DFS but not with the NPI which is a score composed of the best prognostic indicators known today. In contrast to this, ACC was found to be closely and linearly associated to the known prognostic factors. This may suggest that the apoptotic cell counts done on routine sections may be used as a part of prognosis assessment in infiltrating ductal carcinoma.