Parapharyngeal Neoplasm of Mixed Mesenchymal and Neuroepithelial Origin

Shuangshoti, Samruay; Chutchavaree, Amnuay

doi:10.1001/archotol.1980.00790300049010

Cited by 21 publications

(6 citation statements)

References 11 publications

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“…Of these previously reported cases, 15/21 were in children Ͻ3 years old, most often paratesticular with the head and neck region being the second most common site, and 13/18 occurred in males (gender not reported in 3 cases). Five of six cases not occurring in those Ͻ3 years old were adults (Ͼ20 years old), all of whom were from Southeast Asia [2,10,13]. Often, the "EM" in these adults had various other malignant elements (non-neuro/muscular).…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

et al. 2000

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Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants; 10 were < or =3 years old and 5 were > or =6 years old. No male predilection was observed; 7 were female. The originating institutional diagnoses were; RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually > or =5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle +/- neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm.

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Section: Discussionmentioning

confidence: 99%

Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

et al. 2000

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“…We suggest the neural crest, an ectomesenchymal structure [ 11,14,22], as the origin of our patient's median nerve malignant mesenchymoma. Aberrant differentiation of mesenchymal elements of the neural crest into malignant fibroblasts, Schwann cells, and adipose tissue would create a mixed nerve sheath sarcoma and liposarcoma.…”

Section: Discussionmentioning

confidence: 73%

“…Aberrant differentiation of mesenchymal elements of the neural crest into malignant fibroblasts, Schwann cells, and adipose tissue would create a mixed nerve sheath sarcoma and liposarcoma. The Schwann cell as a mesenchymal-type cell has been discussed [4, 11,14]. Neuroepithelial tumors would be formed when the ectodermal part of the neural crest becomes neoplastic.…”

Section: Discussionmentioning

confidence: 99%

Malignant mesenchymoma of median nerve: Combined nerve sheath sarcoma and liposarcoma

Shuangshoti¹,

Benjavongkulchai²,

Chittmittrapap³

1984

Journal of Surgical Oncology

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A malignant mesenchymoma consisting of nerve sheath sarcoma and liposarcoma occurred in the right median nerve of a 29-year-old man who did not have stigmata of von Recklinghausen neurofibromatosis. It produced pain at the affected part, referred pain to the palm, and weakness of muscles supplied by branches of this nerve. The tumor was first excised 1 month after its initial manifestation, but recurred twice and eventually metastasized to the lung within 19 months. It is suggested that aberrant differentiation of the mesenchymal component of the neural crest, the ectomesenchymal structure, has created this combined sarcoma of nerve sheath and liposarcoma, forming a malignant mesenchymoma.

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“…The ectomesenchymoma of soft tissue appears to be a related malignancy of the migratory neural crest and is composed of primitive neuroblasts and/or ganglion cells with a population of rhabdomyoblasts or other malignant mesenchy-ma1 elements. [21][22][23][24] The present neoplasm could be designated as an ectomesenchymoma instead of neuroblastoma with focal rhabdomyomatous differentiation. The occurrence of multiple lines of differentiation in this tumor is not surprising in light of the reported presence of heterologous elements in hepatoblastomas, 25 pancreatoblastomas, 25 and Wilms' tumors.…”

Section: Discussionmentioning

confidence: 99%

Rhabdomyosarcomatous differentiation in a neuroblastoma: A potential pitfall in the cytologic diagnosis of small round‐cell tumors of childhood

Layfield

Glasgow

1991

Diagnostic Cytopathology

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Rhabdomyoblasts demonstrating immunoreactivity for muscle-specific actin, desmin, and myoglobin were identified in smears obtained by aspiration from a large retroperitoneal mass in a 14-mo-old girl. Following a tentative diagnosis of a rhabdomyogenous neoplasm, retroperitoneal exploration and adrenalectomy demonstrated a stromal poor neuroblastoma with extensive rhabdomyogenous differentiation. The presence of a subpopulation of rhabdomyoblasts was not diagnostic of rhabdomyosarcoma when obtained by fine-needle aspiration from a retroperitoneal tumor composed predominantly of primitive small round cells.

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Parapharyngeal Neoplasm of Mixed Mesenchymal and Neuroepithelial Origin

Cited by 21 publications

References 11 publications

Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV

Malignant mesenchymoma of median nerve: Combined nerve sheath sarcoma and liposarcoma

Rhabdomyosarcomatous differentiation in a neuroblastoma: A potential pitfall in the cytologic diagnosis of small round‐cell tumors of childhood

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