Ovarian transposition before pelvic irradiation: indications and functional outcome.

AIM: To investigate the indications and effectiveness of ovarian transposition before pelvic irradiation. METHODS: This was a retrospective analysis of patients with malignancies who underwent ovarian transposition before pelvic irradiation. The collected data included age of patient, type and stage of cancer, details of irradiation treatment, and clinical and biochemical parameters of ovarian function during the period of follow-up. RESULTS: Fourteen adult and four pediatric patients with different types of cancers underwent ovarian transposition during the study period. The common tumor types for the adult patients were cervical cancer (n = 4), rectal cancer (n = 4) and medulloblastoma (n = 3), and for pediatric patients was medulloblastoma (n = 2). The mean age for adult patients was 31 years (range, 21-40) and for pediatric patients was 7 years (range, 4-10). Of the adult patients, 10 had adjuvant chemotherapy and four had neoadjuvant chemotherapy added to their radiotherapy program. All pediatric patients received adjuvant chemotherapy. Thirteen of 14 (92.85%) adult patients had normal serum level of follicle-stimulating hormone (FSH; ≤12 IU/L) and E2 (>50 pg/mL). Only one patient had premature menopause. All pediatric patients demonstrated a normal serum level of FSH (<12) and E2 for their age at 3 and 6 months after completion of their treatment. The mean follow-up was 42 months (range, 34-50). CONCLUSION: Ovarian transposition is an effective procedure for the preservation of ovarian function. Young patients with non-hormone-dependent pelvic tumors should be offered a laparoscopic ovarian transposition before the start of pelvic radiotherapy.

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy.

BACKGROUND: The care of rhabdomyosarcoma (RMS) is complex due to its multimodal nature. By following standard protocols with acceptable toxicity and building local expertise, better outcome should be achievable. PROCEDURE: A retrospective study was conducted of records of patients (n = 45; 31 males; median age 26 months) with RMS treated at King Hussein Cancer Center in Jordan from January 2004 to December 2008. Patient demographics, tumor characteristics, risk stratification, treatment plan, and outcomes were studied. In June 2006, the cyclophosphamide dose was lowered from 2.2 g/m(2) to 1.2 g/m(2) per cycle because of the significant toxicity with higher dose. Survival rates, hematological toxicities, period of hospitalization due to febrile neutropenia (FN), and response rate at week 12 of treatment were compared between low- and high-dose cyclophosphamide groups. RESULTS: Four-year progression-free survival (PFS) and overall survival (OS) rates were 61% ± 7.5% and 72% ± 6.9%, respectively. There was a significant difference in outcome by risk group in 4-year PFS (low-risk, 88% ± 12%; intermediate-risk 63% ± 9.3%; high-risk, 14% ± 13%; P = 0.0001) and OS (low-risk, 88% ± 12%; intermediate-risk 79% ± 7.5%; high-risk, 17% ± 15%; P = 0.0011). There was significant reduction in hematological toxicities, incidence of FN, and period of hospitalization for FN in patients given low-dose cyclophosphamide but no significant difference in PFS between low- and high-dose cyclophosphamide groups. CONCLUSIONS: Survival rates of patients with RMS in some developing countries can be improved by following or modifying evidence-based approaches successful in developed countries and establishing multidisciplinary strategies. Therapy intensity should be increased in developing countries only when evidence supports its utility.

Chromosome 3q29 deletion with gastrointestinal malformation: a case report.

INTRODUCTION: Most chromosome 3 deletions are associated with neuro-developmental and eye abnormalities. Here, we report a rare and unusual multiple congenital abnormality, including ano-rectal malformation, in conjunction with chromosome 3q29 segment deletion, which has not previously been reported. CASE PRESENTATION: A three-month-old female Jordanian baby presented with an absent anus and corneal opacities and was referred for further management after a diverting colostomy operation at the age of one day. CONCLUSION: Chromosome 3q29 deletion is associated with additional abnormalities to neurological ones, such as ano-rectal malformations. We need to investigate a patient fully to find such hidden clinical features.

Inflammatory myofibroblastic tumor causing unexplained anemia in a toddler: a case report.

INTRODUCTION: Inflammatory myofibroblastic tumor is a very rare benign tumor in children that mimics malignant tumors in its aggressiveness locally and by the possibility of recurrence after surgical resection, and causing anemia of chronic disease, which is a decrease in hemoglobin 1 to 2 g/dL below normal level in a patient with chronic illness. CASE PRESENTATION: A 32-month-old boy from Libya presented with microcytic hypochromic anemia. He had been treated in three countries and five centers without response to medical therapy. He was investigated at our center and found to have a mass in the colon causing intermittent intussusception and bleeding. He was treated surgically, and his condition improved dramatically. The pathology report proved a diagnosis of inflammatory myofibroblastic tumor. CONCLUSION: We report a case of an unusual tumor of the gastrointestinal tract causing chronic anemia not responding to medical treatment, and discuss the characteristics of inflammatory myofibroblastic tumor. In our case, we stress the involvement of a multidisciplinary team in treating such a patient who presents with common symptoms and signs but in whom there has been no response to any of the measures and treatment protocols.

Distinct features of teratoid Wilms tumor.

Teratoid Wilms tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. Typically, histopathologic examination shows a mixture of mature squamous and mucus-producing columnar epithelium and a prominent component of mature adipose tissue. The treatment of this tumor has not been established because of its rarity with some reports doubting the value of chemotherapy. We report 3 new cases and review the 23 previously reported cases that we were able to identify. The first case was a 2-year-old boy with a huge tumor that occupied most of the abdominal cavity. The second case was a 5-year-old girl with a metastatic tumor in association with horseshoe kidney and is the first case of teratoid Wilms tumor with brain metastasis. The third case was an 11-month-old girl with bilateral involvement. Imaging studies of our patients were similar to those reported in the literature with areas of fat attenuation and calcification. Failure to respond to preoperative chemotherapy was seen in our patients and in most previously reported cases that were treated with preoperative chemotherapy. We recommend treating this entity with protocols designed for Wilms tumor and to conduct further basic research to understand its biologic nature.

INI1 (BAF 47) immunohistochemistry is an essential diagnostic tool for children with hepatic tumors and low alpha fetoprotein.

Malignant rhabdoid tumor (MRT) of the liver is a rare malignancy with grave prognosis. This entity should be considered in the differential diagnosis of any aggressive liver tumor with low levels of alpha fetoprotein. We report 2 cases of hepatic MRT presenting in infancy. In these 2 cases, we show that loss of INI1 facilitates making the correct diagnosis of primary hepatic MRT utilizing BAF 47 (INI1 gene product) immunostains. Difficulty encountered in making this rare diagnosis, including the need for repeated biopsies, can be avoided if MRT is considered in the differential diagnosis early on and BAF 47 immunohistochemistry is ordered.

From upfront nephrectomy to preoperative chemotherapy and back: a single institution experience in the treatment of Wilms tumor.

BACKGROUND: Over the past decades, 2 different approaches for the treatment of Wilms tumor have emerged: upfront nephrectomy (UN) and preoperative chemotherapy (PC), with adjuvant treatment adjusted to stage, histology, and chemotherapy response. METHODS: In July 2005, we switched our strategy from UN to PC. This study is a retrospective review of patients treated at our institution between January 2003 and October 2007. RESULTS: Thirty-six children (20 males) with Wilms tumor were studied. Median age was 3.45 years (range: 0.3 to 15.8 y). Nineteen patients (53%) were treated according to the International Society of Paediatric Oncology 93-01/German Pediatric Oncology Hematology, Group protocol (PC group) and 17 (47%) according to the National Wilms' Tumor Study-5 (UN group). UN group received more radiation dose and less cumulative doses of doxorubicin. The 3-year event-free survival and overall survival estimates for the whole group were 86% and 89%, respectively. Survival estimates were similar in both groups. CONCLUSIONS: The use of PC reduced the use of radiation; however, patients treated using the SIOP 93-01/German Pediatric Oncology Hematology Group protocol received higher cumulative doses of doxorubicin; these doses were believed to be high in this young group of patients with potential for long-term toxicity. Although selecting a specific protocol for Wilms tumor is important, the development of surgical expertise and referral to specialized centers takes priority.