Juvenile Hyaline Fibromatosis: A 10-year Follow-up.

Juvenile hyaline fibromatosis (JHF) is a rare hereditary disease with an autosomal recessive transmission. JHF is characterized by papulonodular skin lesions, osteolytic bone lesions, flexural joint contractures, and gingival hyperplasia and usually diagnosed in infancy or early childhood. JHF is thought to be a disorder of collagen metabolism and characterized by homogenous amorphous eosinophilic material and fibrous tissue. We report the case of a 14-year-old male child with multiple papulonodular skin lesions, progressive flexion contractures of joints, and severe gingival hyperplasia, with a 10-year follow-up. Although the lesions were totally removed thrice during the last 10 years, they recurred rigorously.

Virginal Breast Hypertrophy: Different Presentations of 2 Cases and the Role of Tamoxifen as an Adjuvant Therapy.

BACKGROUND: Virginal breast hypertrophy is a rapid and massive enlargement of one or both breasts. There are several proposed causes and treatment options for virginal breast hypertrophy, but the investigations to support these theories are lacking. CASES: We report two premenarchal girls with virginal breast hypertrophy who presented as different clinical cases. After their surgical interventions, their clinical courses were followed for more than 2 years with tamoxifen as an adjuvant therapy. SUMMARY AND CONCLUSION: Breast size and shape disorders can be a disturbing cosmetic problem for adolescents who worry about their body image. A combination treatment of breast reduction surgery and tamoxifen is reasonable and can eliminate the need for repeated surgeries for girls with virginal breast hypertrophy.

Giant cell tumor of the metacarpal bone in children: free osteoarticular metatarsal transfer: case report.

We present a successful total resection of metacarpal bone and nonvascularized joint transfer in a giant cell tumor of the fourth metacarpal bone in a 13-year-old girl. At the 6-year follow-up, a good functional outcome was achieved, with 85° range of motion of the metacarpophalangeal joint and no clinical or radiographic evidence of tumor recurrence and no signs of degeneration of the joint.

Adenoid cystic carcinoma of the breast: A case report and literature review.

Adenoid cystic carcinoma (ACC) is a rare malignant tumor of the breast that occurs in <0.1% of all patients diagnosed with breast cancer. The mean patient age at the time of diagnosis is 50-60 years. Typically, the tumor presents as a subareolar mass or as pain in the breast. While the radiological appearances of ACC are generally non-specific, the diagnosis can be made on fine-needle aspiration cytology. In the present study, a 58-year-old female patient was admitted to the Department of Radiation Oncology (Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey) with complaints of pain in the upper outer quadrant of the right breast. An excision biopsy of a lump in the upper outer quadrant revealed ACC, and perineural invasion was present. Subsequently, the patient underwent breast conservation surgery and sentinel lymph node dissection. Pathology from the second surgery depicted ACC in the form of microscopic foci around the initial surgical cavity, with two reactive sentinel lymph nodes and the closest negative margin at 2 mm. The patient was treated with radiotherapy following the surgery. No recurrence and metastasis were found after 20 months of follow-up. In conclusion, mammary ACC is a rare malignant neoplasm of the breast. Although surgery is the main treatment, the optimal adjuvant treatment of ACC of the breast has not yet been determined due to its low incidence.

[Chondroblastoma in the anterior cruciate ligament origo: a case report]. / Ön çapraz bag yapisma yerinde kondroblastom: Olgu sunumu.

Chondroblastoma is a rarely seen cartilage originated tumor. It is mostly localized in the epiphysis of long bones. In this article, we present an 18-year-old male case in whom the tumor was located in the right distal femoral lateral condyle and destroyed anterior cruciate ligament origo. The tumor was curetted and the cavity was filled with cement. Anterior cruciate ligament resection was mandatory for this treatment. The patient had no complaint in the postoperative period.

Primary posterior mediastinal Burkitt lymphoma.

A 14-year-old boy admitted to our clinic with a 20-day history of fever, cough and respiratory distress. Mediastinal enlargement was observed on chest radiograph. Computed tomography of the chest demonstrated a large posterior mediastinal mass. The histopathological examination of the mass revealed Burkitt's lymphoma. We report this case because primary posterior mediastinal involvement in Burkitt lymphoma is extremely rare in childhood.

Penile metastasis from adenocarcinoma of the prostate in a patient with colorectal carcinoma.

Skin metastasis secondary to cancer of the prostate is rare and the prognosis is poor. A 65-year-old male patient diagnosed with metastatic colon carcinoma presented with polyuria and subcutaneous nodular mass on dorsal side of the corpus penis. The serum prostate specific antigen (PSA) level was 111.1 ng/mL and therefore the patient underwent transperineal prostate biopsy. Pathology reported adenocarcinoma of the prostate. The subcutaneous nodular lesion on the penis was totally excised and removed. Immunohistochemical examination of the excised mass was carcinoembryonic antigen (CEA) negative and PSA positive. Taking all these findings into consideration, the patient was diagnosed with prostate cancer that had metastasized to the penis. FOLFOX-4 chemotherapy regime in addition to bicalutamide and goserelin acetate was administered to the patient who also had metastatic colon cancer.

Intraarticular fibroma of the tendon sheath arising from the infrapatellar fat pad in the knee joint.

We report a rare case of fibroma of the tendon sheath arising from the fat pad in the knee. To our knowledge, only four previous cases of fibroma of the tendon sheath in the knee have been reported in the English literature. In our case, magnetic resonance imaging showed low signal intensity in the mass on T1- and T2-weighted images. The mass was excised by mini arthrotomy.

An unusual case of an aldosterone-producing adrenocortical adenoma presenting with rhabdomyolysis.

Rhabdomyolysis is a rare presentation of hypokalemia, although muscle weakness is a well known manifestation of marked hypokalemia. Here, we report a case of primary hyperaldosteronism due to unilateral aldosterone-producing adenoma in a 14 year-old girl who developed rhabdomyolysis following hypokalemia. To our knowledge, this is the first case of adrenocortical adenoma presenting with rhabdomyolysis in a child.

Woringer-Kolopp disease coexpressing CD4 and CD8 treated with radiation therapy: a case report.

We present an extremely rare case of Woringer-Kolopp disease with CD4+/CD8+ immunohistochemical features in a 27-year-old male patient, which involved the right gluteal region continuing down to the lower thigh. Although the lesions had been present for 15 years, the disease abruptly changed its indolent clinical behavior in the last 3 months and gained local aggressiveness without features of dissemination. The patient was successfully treated with 6 MeV electron-beam radiotherapy and the radiation portal was determined to be the gross lesion plus 1-cm margins around the lesion. Radiation therapy was administered with a hypofractionated dose schedule of 2.8 Gy per fraction in 13 fractions, 5 days a week, to a total of 36.4 Gy. No radiation-induced toxicity was reported during therapy. The patient's follow-up was ordinary with no recurrence after a follow-up period of 38 months and the cosmetic result was excellent.

A case of juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare, autosomally-recessive disease characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. Its onset is in infancy or early childhood. The most commonly affected sites are the nose, chin, ears, scalp, back and knees. The accumulation of an amorphous, hyaline material is typical in the skin and the other organs. Herein, we report a 14-month-old boy who presented with confluent pink papules on the paranasal folds and the chin, and nodular lesions on the periauricular and perianal regions. He had gingival hypertrophy and contractures of the shoulders, knees and elbows. He also had third-degree consanguineous parents. Histopathological studies confirmed the diagnosis of JHF with the presence of increased numbers of fibroblasts embedded in a hyalinized connective tissue stroma.

Juvenile hyaline fibromatosis: a case report.

Juvenile hyaline fibromatosis ( JHF ) is a rare autosomal recessive disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and bone lesions. The skin lesions may consist of multiple large tumors, commonly on the scalp and around the neck, and small pearly, pink papules and plaques on the trunk, chin, ears, and around the nostrils. Here, we report a 2-year-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hyperplasia. The lesions were totally excised and clinicopathological diagnosis was JHF.

Castleman disease: a case with atypical presentation.

Castleman disease is a benign lymphoproliferative disorder characterized by enlarged lymph nodes. In children the disease is rare, usually localized, and asymptomatic. Resection of the node is almost always curative. A case is reported that was diagnosed as hyaline vascular-type Castleman disease at 1 year of age. The disease recurred from infraclavicular region in addition to primary site, even though total excision was performed. Although the disease is mullticentric after recurrence, the patient has no systemic symptoms.

Shunt dysfunction due to calcification of a ventriculo-peritoneal shunt: a case report.

Calcification and related dysfunction of ventriculo-peritoneal shunts are rare events in neurosurgical practice. Shunt calcification causes shunt dysfunction in two ways, namely disconnection and obstruction. We present a 16-year-old girl with shunt malfunction due to disconnection secondary to calcification. The shunt tubing fractured during attempted removal and some of the remaining components, including the ventricular catheter, had to be left in situ. The shunt was revised and the patients symptoms resolved. Replacement of the calcified and perished shunt components with a new shunt is essential. Aggressive surgical manipulation for removal of the remaining shunt components is not advisable as this may increase morbidity and mortality.

Detection of chromosomal aberrations in prostate cancer by fluorescence in situ hybridization (FISH).

PURPOSE: Fluorescence in situ hybridization (FISH) is a powerful tool for quantitative analysis of chromosomes and genes and can be applied in a variety of specimens, including cell cultures, isolated nuclei from fresh and fixed tissues, and histological tissue sections. For detection of numerical chromosome aberrations, we examined prostatic cancer samples at our department. In addition, we also observed primary and secondary aberrations taking part in the initiation and progression of tumours. MATERIALS AND METHODS: FISH using chromosome-specific alpha-satellite DNA probes for chromosomes 7, 8, 9, 10, 17, X and Y was performed on 19 prostatic cancer and 19 benign prostatic hyperplasia (BPH) samples obtained from transurethral resection (TUR) and archival paraffin-embedded blocks. RESULTS: Numerical aberrations were observed in 41% of the tumours studied. A range of aberrant copy numbers of chromosome 9 (68%), 7 (63%), 8 (58%), 17 (37%), Y (32%) and 10 (26%) was observed. We did not observe significant aberrations in BPH samples. In prostate cancer patients, chromosomes 7 (47%), 8 (58%) and 9 (63%) were monosomic by FISH. Monosomy 8 and 9 were significant differences (p>0.05) between prostate cancer and BPH patients. CONCLUSIONS: FISH analysis could be observed an one of strongest methods of analysis in detecting numerical aberrations of individual chromosomes with application to paraffin-block samples, metaphase and, interphase nuclei. To our knowledge, this analysis is firstly studied in Turkish patients. Therefore, results of this analysis may be important for Turkish patients.