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Shih-Chiang Huang, Rita Alaggio, Yun-Shao Sung, Chun-Liang Chen, Lei Zhang, Yu-Chien Kao, Narasimhan P Agaram, Leonard H Wexler, Cristina R Antonescu
Malignant ectomesenchymoma (MEM) is an exceedingly rare pediatric sarcoma with a predilection for infants and young children and is composed of dual malignant mesenchymal and neuroectodermal components. Microscopically, MEM displays areas of rhabdomyosarcoma (RMS) with intermixed neuronal/neuroblastic foci. The molecular alterations associated with MEM and its relationship with embryonal RMS (ERMS) and malignant peripheral nerve sheath tumor (MPNST) have not yet been elucidated. In this study we used whole-transcriptome sequencing in 2 MEM index cases with available frozen tissue, followed by screening of the identified genetic abnormalities in 5 additional cases...
July 2016: American Journal of Surgical Pathology
Wei-Tang Kao, Yi-Te Chiang, Kai-Yi Tzou
Malignant ectomesenchymoma (MEM) which is derived from the remnants of migratory neural crest cells (ectomesenchyme) is a rare and rapidly progressing tumor consisting of neuroectodermal and mesenchymal neoplastic elements. This tumor occurs mostly in children and adolescents, but rarely in adults. We report a 34-year-old male with left paratesticular malignant ectomesenchymoma who received radical orchiectomy and was followed by post-operative flare-up of lung metastasis within 2 weeks. We present the overall treatment strategies for this extremely rare tumor and related findings...
September 2015: Urology Case Reports
Rita Alaggio, Cheryl M Coffin
This review discusses the history of the classification of soft tissue sarcomas in children and adolescents, the current transition toward integration of morphology and molecular genetics as new entities emerge, and future perspectives.
November 2015: Pediatric and Developmental Pathology
Yao Kun, Zejun Duan, Xi Mei, Ying Xu, Jiuzhou Li, Shouwei Li, Xueling Qi
Malignant ectomesenchymoma is a rare tumor that contains both ectodermal and mesenchymal elements. So far, only 7 patients with a manifestation in the cerebrum (with confirmed clinicopathological data) have been reported. A 4-year-old girl was present at our hospital with a 3-week history of intermittent sudden dizzy with no apparent cause. MRI showed an irregular enhanced lesion in the left frontal-parietal lobe and lateral ventricle with peripheral gadolinium-enhancement with a significant surrounding edema...
2015: International Journal of Clinical and Experimental Pathology
A Nael, P Siaghani, W W Wu, K Nael, Lisa Shane, S G Romansky
Pediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from pluripotent embryologic migratory neural crest cells. We report a 4-year-old boy who initially presented with a pelvic mass and inguinal lymphadenopathy at 6 months of age. Inguinal lymph node biopsy revealed a distinct biphasic tumor with microscopic and immunophenotypic characteristics diagnostic for both alveolar rhabdomyosarcoma and poorly differentiated neuroblastoma...
2014: Case Reports in Pediatrics
Katherine A VandenHeuvel, David F Carpentieri, Jie Chen, Kar-Ming Fung, David M Parham
Soft-tissue tumors known as "triton" tumors are rare lesions containing neural tissue and skeletal muscle at varying levels of maturity and malignant potential. Benign triton tumors, also called "neuromuscular choristomas" or "neuromuscular hamartomas," consist of neural tissue containing mature skeletal muscle in intimate relationship with peripheral nerve. These tumors are rare in the head and neck in children. Ectomesenchymomas are similar tumors consisting of a malignant mesenchymal component, usually embryonal rhabdomyosarcoma, and a neuroectodermal component represented by mature ganglion cells or primitive neuroblastic/neuroectodermal foci (primitive ectomesenchymoma)...
May 2014: Pediatric and Developmental Pathology
Channappa N Patil, Sanju Cyriac, Urmila Majhi, Rejiv Rajendranath, T G Sagar
Malignant ectomesenchymomas are rare tumors. This tumor affects predominantly young children. Most common site is head and neck. A multi modality approach should be appropriate for this soft-tissue tumor. We present a 43 year old female with malignant ectomesenchymoma of the nasal cavity.
October 2011: Indian Journal of Medical and Paediatric Oncology
Tobias M Dantonello, Ivo Leuschner, Christian Vokuhl, Stefan Gfroerer, Andreas Schuck, Stefanie Kube, Michaela Nathrath, Benedikt Bernbeck, Peter Kaatsch, Niklas Pal, Gustaf Ljungman, Stefan S Bielack, Thomas Klingebiel, Ewa Koscielniak
BACKGROUND: Malignant ectomesenchymoma (MEM) is a soft tissue tumor with heterologous rhabdomyoblastic components believed to arise from pluripotent migratory neural crest cells. To date merely 50 cases have been published and the knowledge about the course of disease and optimal treatment is limited. METHODS: Six patients with MEM were registered 1996-2009. The diagnosis was confirmed according to current criteria. Their treatment and outcome was analyzed. RESULTS: The median age of the three females and three males was 0...
February 2013: Pediatric Blood & Cancer
A S Vinck, B Lerut, R Hermans, S Nuyts, R Sciot, M Jorissen
PROBLEM: A 36-year-old woman presented with a feeling of pressure in the right orbit and proptosis of the right eye after a "common cold". METHODOLOGY: Computed tomography (CT) of the maxillofacial region revealed, and endoscopy confirmed, a mass in the right ethmoid sinus, eroding the lamina papyracea and extending into the orbit. Pathology of multiple biopsies revealed a nasal neoplasm composed of neuroectodermal and mesenchymal neoplastic elements, suggestive of a malignant ectomesenchymoma (MEM)...
2011: B-ENT
Sinead Howley, Deirdre Stack, Thomas Morris, Michael McDermott, Michael Capra, David Betts, Maureen J O'Sullivan
Ectomesenchymoma is a rare mesenchymal malignancy occurring mainly in the pediatric population. The hallmark diagnostic features are a combination of sarcoma, usually rhabdomyosarcoma (RMS) with admixed ganglion cells. The lesion arises either in soft tissues or the cranial cavity, and outcomes vary considerably. Current knowledge about the genetics and biology of ectomesenchymoma is extremely limited with only 4 published karyotypes, showing overlaps only in trisomies 2, 8, and 11. Here, we describe a case with genetic findings that, in conjunction with preexisting observations, offer some additional insights into the genetic aberrations of ectomesenchymoma...
February 2012: Human Pathology
Dana L Altenburger, Aaron S Wagner, Don E Eslin, Gary S Pearl, Jogi V Pattisapu
Malignant ectomesenchymoma is a rare tumor arising from mature ganglion cells with immature myogenous elements, with only 4 pediatric intracranial cases having been previously reported. The authors report a rare case of intracranial malignant ectomesenchymoma originating from the falx cerebri in a 10-year-old boy. The patient presented with a 2-week history of headache, nausea, and blurry vision, with mild lateral gaze diplopia. A CT scan revealed a solitary 7.2 × 3.8-cm dural-based mass that extended along the falx...
January 2011: Journal of Neurosurgery. Pediatrics
Wen-Ping Yang, Liang Feng, Yan Wu, Yin Zou, Song-Tao Zeng, Hua-Sheng Zhong, Hong-Yan Xu, Qiang Xiao
No abstract text is available yet for this article.
October 2009: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Giuseppe Floris, Maria Debiec-Rychter, Agnieszka Wozniak, Elisabetta Magrini, Guidalberto Manfioletti, Ivo De Wever, Giovanni Tallini, Raf Sciot
Malignant ectomesenchymoma (MEM) represents a heterogeneous group of tumors, most likely originating from pluripotent primitive neural crest cells. In this report, we present an 8-month-old infant boy with an MEM on the left scrotum. Retrospective review of the incision biopsy showed the presence of a few ganglion cells in an otherwise classic embryonal rhabdomyosarcoma (RMS), whereas in the resection specimen after chemotherapy the combined RMS and ganglioneuroma components were very obvious. Cytogenetic analysis of the residual lesion showed an abnormal karyotype, 49, XY, +2, -6, +11, +20, +mar, with a hyperploidy in a subset of cells...
December 2007: Diagnostic Molecular Pathology: the American Journal of Surgical Pathology, Part B
B K Kleinschmidt-DeMasters, Mark A Lovell, Andrew M Donson, C Corbett Wilkinson, Jennifer R Madden, Steven O Addo-Yobo, Kevin O Lillehei, Nicholas K Foreman
Gene microarray has been used to identify prognostic markers and genes of interest for therapeutic targets; a less common use is to show possible histogenetic relationships between rare tumor types and more common neoplasms. Intracranial malignant ectomesenchymoma (MEM) is a pediatric tumor postulated to arise from neural crest cells that contain divergent neuroectodermal and mesenchymal tissues, principally mature ganglion cells and rhabdomyosarcoma (RMS). We investigated a case of MEM by molecular, cytogenetic, and gene array analyses and compared results with our previously unpublished series of 51 pediatric tumors including conventional RMS, Ewing sarcoma (EWS), medulloblastoma (MED), atypical teratoid rhabdoid tumor (ATRT), and malignant peripheral nerve sheath tumor (MPNST); the latter is a sarcoma also with potential for divergent differentiation...
June 2007: Acta Neuropathologica
Massimiliano Cergnul, Stefano La Rosa, Mauro Bandera, Paolo Battaglia, Maurizio Bignami, Paolo Castelnuovo, Carlo Capella, Graziella Pinotti
No abstract text is available yet for this article.
April 2007: Lancet Oncology
Ibrahim Bayram, Göksel Leblebisatan, Hacer Yildizdaş, Ulkü Tuncer, Melek Ergin, Nejat Narli, Atila Tanyeli
Malignant ectomesenchymoma is a rare tumor reported in head-neck, abdomen and perineal regions. It consists of mesenchymal and neuroectodermal elements. In this tumor group, neoplastic cells are differentiated into neuronal cells. It also has at least one malignant mesenchymal element, generally rhabdomyosarcoma. In this report we present a neonate with ectomesenchymoma.
October 2005: Turkish Journal of Pediatrics
Elisabeth Weiss, Clemens F Albrecht, Jochen Herms, Julianne Behnke-Mursch, Arnulf Pekrun, Knut Brockmann, Clemens F Hess
UNLABELLED: Malignant ectomesenchymoma is a rare tumour that contains both ectodermal and mesenchymal elements. Only three patients with a manifestation in the cerebrum and clinicopathological data have been reported until now. We present a patient with an intracerebral ectomesenchymoma, review the literature and discuss currently available therapeutic options. In a 10-year-old girl, a left suprasellar temporo-parieto-occipitally localised tumour was diagnosed. The tumour was completely excised macroscopically in two surgical sessions...
June 2005: European Journal of Pediatrics
Orit Oppenheimer, Edward Athanasian, Paul Meyers, Cristina R Antonescu, Richard Gorlick
Malignant ectomesenchymomas, rare and potentially aggressive tumors, occur in children and exhibit mesenchymal and neuroectodermal components. This report describes the first patient diagnosed with a malignant ectomesenchymoma of the hand. The patient was a 17-month-old male who developed a hypothenar mass on his left hand that was surgically excised. Microscopic evaluation revealed ganglioneuroblastic, rhabdomyosarcomatous, and chondrosarcomatous elements. Following excisional biopsy he was treated with cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide...
January 2005: International Journal of Surgical Pathology
Mustafa Kösem, Ibrahim Ibiloğlu, Vedat Bakan, Burhan Köseoğlu
Ectomesenchymoma (EMCH) is a rare tumor that may arise in the brain or soft tissue. This tumor type is defined as a form including ectodermal components represented by neuroblasts or ganglion cells and differentiated mesenchymal structures of various types. The mesenchymal component is most often a rhabdomyosarcoma, but liposarcoma, malignant fibrous histiocytoma, leiomyosarcoma, chondrosarcoma, malignant schwannoma, and osseous elements have also been recorded. We report a case of an abdominal malignant ectomesenchymoma, containing three components, schwannoma, embryonal rhabdomyosarcoma, and ganglion cells, in a four-month-old infant...
January 2004: Turkish Journal of Pediatrics
D Brehmer, H M Overhoff, A Marx
To the best of our knowledge, a malignant ectomesenchymoma of the nose has not yet been reported. This rare tumour is composed of a mesenchymal element--usually a rhabdomyosarcoma--and a neuroectodermal element, often a neuroblastoma or a malignant schwannoma. Most malignant ectomesenchymomas have been found in children (mainly boys), but 5 adult cases, including our case, have also been reported. A 62-year-old man presented with a nasal obstruction on the right side of the nose. Diagnostic nasal endoscopy revealed a mass in the dorsal part of the right nasal cavity, which was treated by endoscopic surgery...
January 2003: ORL; Journal for Oto-rhino-laryngology and its related Specialties
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