Malignant Ectomesenchymoma of the Orbit in a Child

Paikos, Peter; Papathanassiou, Miltiadis; Stefanaki, Kalliopi; Fotopoulou, Maria; Grigorios, Spyropoulos; Tzortzatou, Foteini

doi:10.1016/s0039-6257(02)00313-2

Cited by 16 publications

(11 citation statements)

References 37 publications

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“…MEMs usually spread en route circulation to lung or bone, or en route through the lymphatic system to retroperitoneal lymph nodes. 12 To the best of our knowledge, our case is the first with the characteristic of flare-up of lung metastatic lesions after surgical excision of the primary tumor.…”

Section: Discussionmentioning

confidence: 71%

An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis

Kao

Chiang

Tzou

2015

Urology Case Reports

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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.

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Section: Discussionmentioning

confidence: 71%

An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis

Kao

Chiang

Tzou

2015

Urology Case Reports

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“…The presence of the MEM component, in this case with an RMS phenotype, could be explained by the plasticity of the tumour cells, which might have allowed dedifferentiation. We have not been able to find any study to support this hypothesis, however, the prevalence of this phenomena, mostly in a young population, along with a first diagnosis of pure RMS, may be two plausible arguments which strengthen this theory[ 14 , 15 ]. Hence, further studies are needed to confirm/infirm this hypothesis.…”

Section: Discussionmentioning

confidence: 99%

Primary rhabdomyosarcoma: An extremely rare and aggressive variant of male breast cancer

Satala¹,

Jung

Bara³

et al. 2020

WJCC

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BACKGROUND Rhabdomyosarcoma (RMS) of the breast, a mesenchymal neoplasm with skeletal muscle differentiation, is an extremely rare tumour in males, with less than 30 cases published in English-language literature. We report on the first case of a male breast RMS, with an unusual ectomesenchymal/neuroectodermal component. CASE SUMMARY A 55-year-old, previously healthy male, underwent a radical left mastectomy for an ulcerated tumour mass, occupying the breast and left anterior thoracic wall. The biopsy specimen indicated the presence of a tumour with neural origins, namely a peripheral neuroectodermal tumour (PNET). The surgical specimens identified two components. The rhabdomyosarcomatous component (over 70%) was represented by large pleomorphic cells with positivity for desmin, sarcomeric actin and myogenin. The PNET-like ectomesenchymal component, which was admixed with the RMS cells, and was also revealed during the preoperative biopsy, consisted of small cells which expressed neurofilament, neuron specific enolase and CD99. The microscopic examination, along with the immunohistochemical profile, allowed the diagnosis of an RMS, with unusual ectomesenchymal differentiation. The patient refused the postoperative oncologic therapy and died three months after surgery. CONCLUSION In patients with RMS of the breast, the PNET-like ectomesenchymal component increases the diagnosis difficulty, especially in biopsy specimens. This differentiation can be immunohistochemically proven and might highlight the possible development of high-grade sarcoma of the breast from remnants of the embryological ectodermal layer.

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“…MEM being highly aggressive has been managed by gross‐total resection and chemotherapy (often based on a sarcoma protocol). In the majority of cases, RMS being the malignant component, the decision was made to treat the patients with sarcoma‐based therapy . Most cases of extracranial location have been treated according to protocols of intergroup RMS trials .…”

Section: Discussionmentioning

confidence: 99%

“…In the majority of cases, RMS being the malignant component, the decision was made to treat the patients with sarcoma-based therapy. 2,24 Most cases of extracranial location have been treated according to protocols of intergroup RMS trials. 5 However, the right drugs should be chosen based on the MEM components.…”

Section: Post-operativementioning

confidence: 99%

Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature

et al. 2019

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Malignant ectomesenchymoma (MEM) is an exceedingly rare rapidly progressing tumor of soft tissues of the central nervous system, believed to be derived from neural crest cells. The majority of cases have been observed in young children or adolescents. So far only 11 patients with intracranial manifestations (with confirmed clinicopathological data) have been documented. We report the first case of adult intracranial MEM in a 54‐year‐old man who presented with a 4 months history of headache and weakness of right side of the body. Magnetic resonance imaging showed a homogenously enhanced dural‐based lesion in the left fronto‐temporo‐parietal lobe with significant perilesional edema and mass effect. No metastatic disease was identified and the lesion was grossly resected. Histopathological and immunohistochemical examination revealed mature and immature neurons and bizarre astrocytes admixed with a mesenchymal spindle cell (rhabdomyoblastic) component. Specific risk factors that contribute toward the development of MEM are unknown. Due to the scarcity of reported cases the role of adjuvant therapy is unclear.

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Malignant Ectomesenchymoma of the Orbit in a Child

Cited by 16 publications

References 37 publications

An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis

An Adult Paratesticular Malignant Ectomesenchymoma With Post-operative Flare-up of Lung Metastasis

Primary rhabdomyosarcoma: An extremely rare and aggressive variant of male breast cancer

Primary intracranial malignant ectomesenchymoma in an adult: Report of a rare case and review of the literature

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