A Triton tumor mimicking sacrococcygeal teratoma

Sönmez, Kaan; Türkyılmaz, Zafer; Karabulut, Ramazan; Kapısız, Alparslan; Eser, Eylem Pınar; Memış, Leyla; Başaklar, A. Can

doi:10.1016/j.jpedsurg.2009.07.042

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…Although most MTT cases are diagnosed in patients under 35 years of age, few cases have been reported in children and adolescents (9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). Main series have shown no gender predilection and, although widely distributed, the head, neck and trunk have been described as the most common locations.…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal malignant triton tumor in an adolescent with Neurofibromatosis type 1

Andión¹,

Buendía²,

Camarena³

et al. 2020

Preprint

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Malignant triton tumor (MTT) is a very infrequent variant of the malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation. Up to 70% of cases are diagnosed in patients with neurofibromatosis type 1 (NF1). It is a highly aggressive pathology with early relapses occurring in up to 50% of patients. Despite multimodal treatment the prognosis is poor, with long term survival rates not exceeding 15%. We present the case of an adolescent male with known NF1 diagnosed with an aggressive retroperitoneal MTT and disseminated pulmonary disease.

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Section: Discussionmentioning

confidence: 99%

Retroperitoneal malignant triton tumor in an adolescent with Neurofibromatosis type 1

Andión¹,

Buendía²,

Camarena³

et al. 2020

Preprint

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“…[4] Three-quarters of sacrococcygeal teratomas are evident at birth, [5] and these represent the majority of malignant lesions in this area. [6] Isolated cases of other malignant lesions in the sacrococcygeal region in this age group have been published, including teratoid Wilms' tumor, [7] malignant Triton tumor, [8] ependymoblastoma [9,10] and neuroblastoma. [11] In the only congenital sacrococcygeal PNET case reported to date, primary excision was incomplete [2] and the patient received dactinomycin and vincristine.…”

Section: Discussionmentioning

confidence: 99%

Congenital sacrococcygeal PNET and chemotherapy

Hawkes

Betts²,

O’Brien

et al. 2012

Indian Journal of Medical and Paediatric Oncology

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We present the case of a congenital localised sacrococcygeal primitive neuroectodermal tumor treated aggressively with surgical resection and modified age-appropriate adjuvant chemotherapy. The conventional combination chemotherapy of vincristine, adriamycin, cyclophosphamide, ifosfamide and etoposide was modified to a regimen including vincristine, adriamicin, cyclophosphamide and actinomycin in order to minimise the predicted toxicity in this age group. Adjuvant “induction” chemotherapy commenced at 4 weeks of age and consisted of four cycles of vincristine, adriamycin and cyclophosphamide at 50%, 75%, 75% and 100% of recommended doses (vincristine 0.05 mg/kg, adriamycin 0.83 mg/kg daily × 2, cyclophosphamide 40 mg/kg) at 3-weekly intervals. This was followed by four cycles of “maintenance” chemotherapy with vincristine (0.025 mg/kg), actinomycin (0.025 mg/kg) and cyclophosphamide (36 mg/kg) at full recommended doses. Cardioxane at a dose of 16.6 mg/kg was infused immediately prior to the adriamycin. Our patient is thriving at 19 months out from end of treatment.

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“…These cases are uncommon in pediatrics. To our knowledge, there have been only 33 reported cases to date (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). Here we present the case of an 8-month-old baby girl with a huge abdominal mass and review the histopathologic, cytogenic, treatment response, and prognostic findings of this rare case of MTT.…”

Section: Introductionmentioning

confidence: 93%