Maysa Al-Hussaini

Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors

Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy.

Closing the survival gap: implementation of medulloblastoma protocols in a low-income country through a twinning program.

Successful twinning initiatives have been reported in childhood leukemia. Pediatric neuro‐oncology requires a complex multidisciplinary approach and the feasibility of similar twinning programs is unknown. Twinning between King Hussein Cancer Center in Amman and the Hospital for Sick Children in Toronto started with e‐mail communications, and subsequently included monthly videoconferences and exchanges between institutions. The outcome of 35 newly diagnosed medulloblastoma patients (22 high‐risk and 13 average‐risk) treated during this period is reported. The 3‐year overall survival for average risk and high‐risk patients was 100 and 81%, respectively. This experience suggests that twinning may facilitate the implementation of multidisciplinary neuro‐oncology programs in low‐income countries. Videoconferencing allows interactive exchanges with a significant learning impact.

Pathologic characteristics of pediatric intracranial pilocytic astrocytomas and their impact on outcome in 3 countries: a multi-institutional study.

Pilocytic astrocytoma (PA) is one of the most common glial neoplasms in the pediatric population, and its gross total resection can be curative. Treatment of partially resected or recurrent tumors is challenging, and the factors associated with increased recurrence risk are not well defined. Identification of favorable and unfavorable factors can lead to a better understanding and management of patients with PA. We studied the pathologic characteristics of 116 intracranial PAs from 4 institutions representing 3 distinct geographic regions to identify factors that may be associated with biological behavior. The study included 65 boys and 51 girls with a median age of 6 years. Median follow-up for all patients was 101 months, during which time 38 patients had recurrence. Progression-free and overall survivals were better in patients who underwent gross total resection. We were not able to identify any differences in pathologic and molecular markers among the 4 institutions from 3 different countries. However, progression-free survival varied significantly among institutions. Sox-2 was the most prevalent stem cell marker in PA, and many tumors showed synaptophysin positivity. BRAF immunostaining was not useful in determining BRAF duplication. BRAF duplication was more typical of posterior fossa tumors. There was a strong correlation between BRAF duplication and pERK immunostaining, suggesting that the RAF/MEK/ERK pathway is active in these tumors. This finding has significant implications given its role in oncogen-induced senescence and possible influence on treatment decisions of subtotally resected tumors.

Closing the survival gap

Successful twinning initiatives have been reported in childhood leukemia. Pediatric neuro‐oncology requires a complex multidisciplinary approach and the feasibility of similar twinning programs is unknown. Twinning between King Hussein Cancer Center in Amman and the Hospital for Sick Children in Toronto started with e‐mail communications, and subsequently included monthly videoconferences and exchanges between institutions. The outcome of 35 newly diagnosed medulloblastoma patients (22 high‐risk and 13 average‐risk) treated during this period is reported. The 3‐year overall survival for average risk and high‐risk patients was 100 and 81%, respectively. This experience suggests that twinning may facilitate the implementation of multidisciplinary neuro‐oncology programs in low‐income countries. Videoconferencing allows interactive exchanges with a significant learning impact.

Embryonal tumor with abundant neuropil and true rosettes: A report of three cases of a rare tumor, with an unusual case showing rhabdomyoblastic and melanocytic differentiation

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is an increasingly recognized entity that belongs to the family of embryonal tumors of the CNS. The authors present three cases of this rare tumor that were encountered at King Hussein Cancer Center, Amman, Jordan. Discussion of the clinicopathological findings is presented along with a recent literature review. Sixteen‐, 57‐ and 30‐month‐old children presented with tumors located in the pineal gland, the right fronto‐ parieto‐temporal region and the cerebellum, respectively. The findings of hypocellular neuropil as well as the characteristic ependymoblastic rosettes were seen. In addition the third case showed an abnormal combination of patterns including melanocytic and rhabdomyoblastic differentiation. The tumors stained positively for synaptophysin in the neuropil and small cell component, while the ependymoblastic rosettes stained for vimentin only. Epithelial membrane antigen and CD99 were negative in all components. One of the cases showed tetraploidy of chromosome 2. All cases exhibited an aggressive course. This is a rare and recently recognized tumor with dismal outcome, and reporting of additional new cases should help in gaining more knowledge about it.

Villoglandular papillary adenocarcinoma of the cervix: a series of 28 cases including two with lymph node metastasis.

Objective To investigate the clinicopathologic features, the management, and the outcome of villoglandular papillary adenocarcinoma (VGPA) of the uterine cervix. Methods A retrospective review of patients’ clinical characteristics, pathology, and the disease management, together with outcome information. Results A total of 28 patients with VGPA were treated. The median age of the patients was 38 years with a range of 26 to 65 years. Sixteen of the 21 patients presented with abnormal bleeding, and 5 patients had an abnormal Papanicolaou (Pap) test result. Nineteen patients had International Federation of Gynecology and Obstetrics stage IB disease, and 5 patients had stage IIB disease. Two of 24 patients, where the lymph node status was known, had positive nodes. Twenty patients underwent different types of radical surgery with or without pelvic radiotherapy, and 8 patients received platinum-based chemotherapy and pelvic radiotherapy with no surgery. The follow-up ranged from 5 to 168 months with a median of 35 months. Twenty–one patients are alive with no evidence of recurrent disease, 5 patients have died because of the disease recurrence, and 2 patients were lost to follow-up. The overall and disease-free 5-year survival for these patients was 82% and 75%, respectively. Conclusion This study confirms the excellent prognosis of VGPA overall compared to the common forms of cervical cancer, but the prognosis is related to stage and pathology. A large multicenter prospective study is warranted to determine the most appropriate treatment for the disease. Until then, a meta-analysis on the subject would be of benefit.

Undifferentiated endometrial carcinoma: A diagnosis frequently overlooked

Undifferentiated endometrial carcinoma (UEC) is a relatively uncommon neoplasm with only few studies published thus far. It has always been a diagnostic challenge because of the lack of proper definition cited in most of the standard textbooks. Recently however, a few studies have highlighted the clinicopathologic features of UEC. The distinctive morphology of UEC was noted by the group from MD Anderson Cancer Center, which enabled them to establish the defining criteria. It appears to be more aggressive than endometrial endometrioid adenocarcinoma, FIGO (International Federation of Gynecology and Obstetrics) grade 3, its main differential diagnosis. Proper recognition of this entity is important owing to its aggressive behavior.

Radiotherapy with concurrent or sequential temozolomide in elderly patients with glioblastoma multiforme

Objective: The objective of this article was to evaluate therapeutic outcomes of elderly patients with glioblastoma multiforme (GBM) treated by surgery followed by combined modality therapy and compare achievable outcomes to those of a younger age population.

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.

Ovarian germ cell tumors with rhabdomyosarcomatous components and later development of growing teratoma syndrome: a case report

IntroductionDevelopment of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically.Case presentationWe report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation.ConclusionThe presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma). In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically.