Malignant Triton Tumor of the Kidney. New Location Not Previously Reported

Gallego, A.; Pontones, J.L.; Ramos, David; Boronat, F

doi:10.1159/000434639

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…This indicated that nerve sheath as well as rhabdomyoblastic components were contained in tumor. [4] MTTs can display as sporadic cases, in young patients, approximately 60% to 70% accompany with neurofibromatosis type 1 (NF1). And deregulation of p53 may play a part in the genesis of MTT.…”

Section: Discussionmentioning

confidence: 99%

A series of 10 malignant triton tumors in one institution

et al. 2019

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Malignant triton tumor (MTT) is an extraordinarily uncommon and aggressive tumor which have poor prognosis. Malignant peripheral nerve sheath tumors with additional rhabdomyoblasts are found in MTT histologically. The prognosis of patients is poor. The goal of our study is to describe the largest number of cases characteristic and outcome, to our knowledge, such a presentation was not described in the English-language literature until now.From 1999 to 2014, 10 patients (5 women and 5 men) with a malignant triton tumor were treated at our institution. All these cases were followed-up and patient charts were analyzed for outcome.In our study, 3 cases of the Malignant triton tumors originate in the head, 2 cases in the joints, 2 cases in the retroperitoneum, 2 cases in the soft tissues of the thoracic wall, and 1 case in the prostate. Neoplasm associated with pain was the main manifestation. Patients have a poor prognosis. Completely surgical excision of the tumor is the only treatment. Additional radiation or chemotherapy show little effect.Malignant triton tumor is a rare sarcoma. The high probability of developing local recurrence and distant metastases could account for its poor prognosis.

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Section: Discussionmentioning

confidence: 99%

A series of 10 malignant triton tumors in one institution

et al. 2019

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“…[ 16 ] Recently, Gallego et al ., reported a case of malignant triton tumor of the kidney. [ 17 ] Malignant triton tumor refers to MPNSTs with rhabdomyosarcomatous differentiation characterized by both neural and skeletal muscle differentiation. [ 18 ] It is extremely rare to have two or more heterogeneous components in a single MPNST.…”

Section: Discussionmentioning

confidence: 99%

Breast metastases from a malignant peripheral nerve sheath tumor of the kidney: An unusual presentation

2016

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Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney). Herein, we describe a case of breast metastases from an MPNST of the kidney without a history of NF-1. The patient was initially diagnosed with a spindle cell neoplasm of the kidney with peripheral nerve sheath differentiation. Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care.

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“…MTT is an aggressive tumor with a poor prognosis, affecting, in most cases, patients under 35 years of age, and the average age of incidence is 31.7 years [7] . The occurrence in pediatric patients is rare [8] , and may be sporadic or occur in conjunction with neurofibromatosis type 1 (NF1) [7] , [9] , with 50–70% of MTTs occurring in those with this genetic condition [10] , [11] . Furthermore, irradiation is considered a risk factor for the development of its sporadic form [9] .…”

Section: Introductionmentioning

confidence: 99%

Malignant triton tumor of the kidney in a child: A case report

Bins

Pinzon

Pereira

et al. 2021

International Journal of Surgery Case Reports

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Introduction Malignant triton tumor (MTT) is an extremely rare variant of the malignant peripheral nerve sheath tumors (MPNSTs) with rhabdomyosarcomatous differentiation, which was first described in 1932 by Mason. MTT affects, in most cases, patients under 35 years of age, and it is usually manifested as a mass that may or not be painful. However, the incidence in pediatric patients is atypical. This tumor presents an aggressive course and limited survival rate, and the prognosis is different between individuals with or without a concomitant diagnosis of neurofibromatosis type 1 (NF1). Currently, the recommended treatment is surgical resection, and adjuvant chemotherapy and radiotherapy, but its efficacy is not yet clear. Presentation of the case A 13-year-old female patient was referred to the pediatric oncology service due to the presence of an abdominal mass and weight loss, initially diagnosed with Wilms' tumor. After extensive investigation, surgical resection, and immunohistopathological evaluation, the diagnosis of malignant triton tumor was confirmed. The patient also underwent cycles of chemotherapy after resection, and is currently awaiting immunotherapy. Discussion and conclusion Malignant triton tumor is extremely rare and difficult to diagnose, especially in children or young people, age groups in which the incidence of the disease is even lower. This may be the reason it is rarely suspected, and it was a great challenge for the clinical care team. It is essential to consider and investigate this possibility of differential diagnosis, as patients diagnosed with this malignant tumor have a low survival rate and poor prognosis.

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Malignant Triton Tumor of the Kidney. New Location Not Previously Reported

Cited by 5 publications

References 9 publications

A series of 10 malignant triton tumors in one institution

A series of 10 malignant triton tumors in one institution

Breast metastases from a malignant peripheral nerve sheath tumor of the kidney: An unusual presentation

Malignant triton tumor of the kidney in a child: A case report

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