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Li−Fraumeni Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
LFS typically causes sarcomas (e.g., osteosarcoma, rhabdomyosarcoma, leiomyosarcoma, orbital liposarcoma, atypical fibroxanthoma, spindle cell sarcoma, histiosarcoma, undifferentiated pleomorphic sarcoma; 25%–30% of cases), brain tumors (e.g., astrocytoma, glioblastoma, medulloblastoma, ependymoma, choroid plexus carcinoma, malignant triton tumor; 9%–16%), adrenocortical carcinoma (ACC; 10%–14%), breast cancer (25%–30%), and other tumors (e.g., colorectal, esophageal, pancreatic, stomach cancers; renal cell carcinoma; prostate, endometrial, ovarian, gonadal germ cell tumors; leukemia, Hodgkin and non-Hodgkin lymphomas; lung cancer; melanoma and non-melanoma skin cancer; non-medullary thyroid cancer). Of these, pre-menopausal breast cancer 28%, soft tissue sarcoma 14%, brain tumor 13%, adrenocortical carcinoma 11%, and osteosarcoma 8% are core cancers most closely associated with LFS. Interestingly, brain tumor, soft tissue sarcoma, and adrenocortical carcinoma commonly appear within the first decade of life, osteosarcoma predominates during the second decade, and breast cancer is often premenopausal. Further, male patients tend to develop brain tumor and hematopoietic and stomach cancers, whereas female patients are often diagnosed with adrenocortical carcinoma and skin cancer. Both males and females are equally affected by soft tissue and bone sarcomas [16–18].
Li-Fraumeni Syndrome
Published in Dongyou Liu, Tumors and Cancers, 2017
Specific cancers related to LFS include sarcomas (e.g., rhabdomyosarcoma, osteosarcoma, leiomyosarcomas, liposarcomas, and histiosarcoma, representing 25%–30% of all LFS-associated tumors), brain tumors (e.g., astrocytoma, glioblastoma, medulloblastoma, ependymoma, choroid plexus carcinoma, and malignant triton tumor, representing 9%–16%), adrenocortical carcinoma (ACC, representing 10%–14%), breast cancer (representing 25%–30%), and other cancers (e.g., colorectal, esophageal, pancreatic, and stomach cancers; renal cell carcinoma; endometrial, ovarian, prostate, and gonadal germ cell tumors; leukemia, Hodgkin and non-Hodgkin lymphomas; lung cancer; melanoma and non-melanoma skin cancers; and non-medullary thyroid cancer) [1,2].
Perineurioma and Malignant Peripheral Nerve Sheath Tumor
Published in Dongyou Liu, Tumors and Cancers, 2017
MPNST with heterologous rhabdomyoblastic (skeletal muscle) differentiation is known as malignant triton tumor (MTT, a name reflecting the ability of triton salamander to regenerate limbs and to grow both neural and muscle components from a transplanted sciatic nerve) [2].
Triton tumor of the orbit
Published in Orbit, 2020
Atanu Barh, Bipasha Mukherjee, Kirthi Koka, Subramanian Krishnakumar
Malignant peripheral nerve sheath tumors (MPNSTs) make up 5–10% of all soft tissue sarcomas.1 Around 8–16% of them are in the head and neck region.2 They are usually derived from Schwann cells or pluripotent cells of the neural crest. They are characterized by alternating hypocellular and hypercellular areas composed of spindle cells arranged in a fascicular growth pattern with variable mitotic rate and tumor necrosis.1Heterologous differentiation can be found in 15% of cases. It includes epithelioid, rhabdomyoblasts, cartilaginous, osseous, smooth muscle, glandular, or rarely liposarcomatous different-iations.3 Rhabdomyomatous differentiation in an MPNST is known as malignant triton tumor (MTT). It accounts for 5% of MPNSTs and has an extremely poor prognosis.4 Areas that are commonly involved include the trunk, head, neck, and extremities.5 There are no reports in the literature of a primary malignant triton tumor involving the orbit. We report a case of malignant triton tumor of the orbit with chondroid and osteoid differentiation originally diagnosed as embryonal rhabdomyosarcoma.
The largest malignant peripheral nerve sheath arising from the gluteal region: case report
Published in British Journal of Neurosurgery, 2023
Safwan O. Alomari, Abdelwahab J. Aleshawi, Raya Marji, Tamara Dawaymeh, Samir M. Albashir, Abdelkarim H. Alomari
Many factors are associated with poor prognosis in MPNST. Large tumor size (>10 cm), higher tumor grade, local recurrence, truncal location, the presence of NF1, and the malignant triton tumor subtype are the main factors associated with a significant adverse impact on disease-specific survival.2,6 Distant metastasis occurs in 20–30% of patients, lung being the most common site followed by bone, liver, peritoneum, and the central nervous system.2 MPNST rarely metastasizes to lymph nodes.