Mediastinal malignant triton tumor: A rare case series and review of literature

Chaudhry, Ikram; Algazal, Thabet; Cheema, Ahsan; Faraj, Aman Al; Malki, Noor Al; Mutairi, Hadi; Abbas, Ahmad; Amr, Samir S.

doi:10.1016/j.ijscr.2019.08.020

Cited by 13 publications

(15 citation statements)

References 21 publications

(23 reference statements)

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“…The head, neck, and trunk regions are reported to be common areas of MTT occurrence, with 20% of MTTs reported in the head and neck, 32% in the trunk, and 24% in the extremities; occurrence in the mediastinum, heart, or lungs is rare and occurs in <10% of cases [ 2 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

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Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location

Zain

Mirchia

Hussien

et al. 2021

Radiology Case Reports

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Malignant triton tumors are an extremely aggressive form of malignant peripheral nerve sheath tumor that display rhabdomyosarcomatous features. While these tumors are extremely rare, they have a much higher incidence in patients with neurofibromatosis-1. We present a case of a 64-year-old male with neurofibromatosis-1 who presented to the hospital with sudden worsening of shortness of breath and dysphagia to solids. Radiological examination revealed a large mass in the anterior mediastinum causing significant narrowing and displacement of the upper trachea and esophagus. Biopsy of the mass, done by interventional radiology, demonstrated features of an MTT. The mass was subsequently resected but without confirmation of tumor-free margins and the patient underwent adjuvant radiation therapy. Repeat radiological examination approximately four months later revealed growing malignancy and new metastases, which eventually contributed to the patient's death seven months after his presentation to the hospital.

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Section: Discussionmentioning

confidence: 99%

“…Chaudry et al. report that, as of 2018, only thirteen cases of mediastinal MTTs have been reported in the literature, of which only five were noted in the anterior mediastinum [12] . The prognosis of MTTs is very poor with a five-year survival rate of only 5%-15% in comparison to MPNSTs, where it is 50%-60% [4] .…”

Section: Discussionmentioning

confidence: 99%

Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location

Zain

Mirchia

Hussien

et al. 2021

Radiology Case Reports

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“…Generally, a de nitive diagnosis for an MTT is based upon observations from histological staining and true positives for S-100 protein, Myo-D1 and Desmin in post-surgical IHC assays. [2][3][8][9] Clinical histories of most patients with MTT cases typically range between several months to more than ten years in all age groups. [15][16] In the two cases exempli ed in this present work, the patient ages were 37 and 41 years, respectively.…”

Section: Discussionmentioning

confidence: 99%

“…[4][5] At present, treatment scenarios for MTTs include radical surgical resection, adjuvant chemotherapy and radiation, with the latter consideration as the standard protocol for MPNST or soft tissue sarcoma. [6][7] In respect of tumor location, MTTs primarily arise in head, neck and trunk regions, but also infrequently observed in lung, mediastinum, abdomen and prostate tissues, [8][9] which invariably culminates with the untimely demise of the infected individual.…”

Section: Introductionmentioning

confidence: 99%

Computed Tomography Imaging Features of Malignant “Triton” Tumor for Its Clinical Diagnosis: Report of Two Cases

Li¹,

Zeng²,

Jiang³

et al. 2020

Preprint

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Background: malignant triton tumors (MTTs) are an extremely rare subtype of malignant periphery nerve sheave tumors (MPNSTs). Clinical diagnosis of MTTs is difficult before surgery due to its low incidence and the lack of knowledge. Therefore, to describe and summarize the computed tomography (CT) imaging characteristics of malignant triton tumors (MTTs) is of great assistance for early and preoperative diagnosis.Case presentation: Two case reports were closely observed in our hospital, with the presumptive diagnosis of MTT by CT scan examination before surgery. The diagnosis of MTT was eventually confirmed by immunochemical (IHC) assay, which verified speculation of CT scans. Huge, irregular, well-circumscribed lobulated mass-like shadows can be observed from these patients by CT scans. Besides, heterogeneity of density within the body of tumor was well-established by CT scans, together with linear septum. Meanwhile, CT scans demonstrated that calcifications were remarkable at the margin of tumor body. Conclusions: Some CT image features from two cases admitted to our hospital were presented as a reference for the preoperative diagnosis of MTTs: (i) enormity of mass-like shadow; (ii) presence of well-circumscribed lobulated shape; (iii) septum within the well-defined mass accompanied with hemorrhage, necrosis and cystic changes as well as calcification, especially within neurofibromatosis type 1 (NF-1) patients.

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“…In both cases, no glandular differentiation was observed. Furthermore, we reported a series of three mediastinal triton tumors, none of them had a glandular component [43].…”

Section: Case Reports In Pathologymentioning

confidence: 99%

Malignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature

AlAli

Amr

2021

Case Reports in Pathology

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Divergent differentiation is a well-known phenomenon in malignant peripheral nerve sheath tumors (MPNST) which occurs approximately in 15% of these tumors, usually towards mesenchymal elements. Differentiation towards epithelial components, however, is quite uncommon, and even exceptionally rare is concomitant mesenchymal and glandular differentiation. To our knowledge, only 14 cases of MPNST with both mesenchymal (rhabdomyoblastic) and glandular differentiation had been reported, and only two of these tumors had frankly malignant glandular components. Herein, we report the third such case. A 26-year-old male, without any of the stigmata of NF1, presented with a 2-year history of pain in his left shoulder and an elbow swelling of six-month duration. The tumor was initially diagnosed clinically as a neurofibroma at a local hospital. The patient underwent excision of the mass there, and pathological examination at that hospital showed the tumor to be MPNST. Six months later, the patient was referred to our hospital, a tertiary care medical center, with recurrent swelling at the same location. Histopathological material from the referral hospital was reviewed, and the tumor was diagnosed as MPNST with rhabdomyoblastic differentiation or malignant triton tumor (MTT) that contained in addition foci of malignant glandular epithelium. The patient refused any surgical intervention. He received three cycles of chemotherapy followed by radiotherapy with excellent response and marked reduction in the size of the tumor. The patient had prolonged survival for 10 years following the initial resection of the tumor.

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Mediastinal malignant triton tumor: A rare case series and review of literature

Cited by 13 publications

References 21 publications

Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location

Malignant triton tumor of the anterior mediastinum: a rare tumor in a rare location

Computed Tomography Imaging Features of Malignant “Triton” Tumor for Its Clinical Diagnosis: Report of Two Cases

Malignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature

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