Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor

Introduction: Immunohistochemistry is a widely used diagnostic technique in pathology, the antibodies used for unvailing a tissue’s origin being made up of proteins, more specifically aminoacids. Malignant peripheral nerve sheath tumors have a distinct immunohistochemistry profile, with great emphas...

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Main Authors: Laura REBEGEA, Elena NICULET, Mihaela CRAESCU, Dorel FIRESCU, Georgiana Bianca CONSTANTIN, Mihaela DUMITRU, Cristina SERBAN, Raul MIHAILOV, Mihaela LUNGU
Format: Article
Language:English
Published: Media Med Publicis 2021-03-01
Series:Modern Medicine
Subjects:
Online Access:https://medicinamoderna.ro/wp-content/uploads/2021/03/Clinical-Histological-Immunohistochemical-12.pdf
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author Laura REBEGEA
Elena NICULET
Mihaela CRAESCU
Dorel FIRESCU
Georgiana Bianca CONSTANTIN
Mihaela DUMITRU
Cristina SERBAN
Raul MIHAILOV
Mihaela LUNGU
author_facet Laura REBEGEA
Elena NICULET
Mihaela CRAESCU
Dorel FIRESCU
Georgiana Bianca CONSTANTIN
Mihaela DUMITRU
Cristina SERBAN
Raul MIHAILOV
Mihaela LUNGU
author_sort Laura REBEGEA
collection DOAJ
description Introduction: Immunohistochemistry is a widely used diagnostic technique in pathology, the antibodies used for unvailing a tissue’s origin being made up of proteins, more specifically aminoacids. Malignant peripheral nerve sheath tumors have a distinct immunohistochemistry profile, with great emphasis concerning those with rhabdomyoblastic differentiation being positive for S-100 protein, myogenin, vimentin, CD99, p63, GFAP, caldesmon, desmin, and p53 and Myo-D1 (the later two revealing the striated muscle differentiation). Materials and methods: A case study concerning a patient suffering from a moderately differentiated (G2) non-keratinizing squamous cell carcinoma of the lung highlighted the development of an undifferentiated sarcomatous proliferation on the left hemithorax, 7 years after the initial external radiotherapy treatment for the malignant lung tumor. Results: The immunohistochemistry analysis of the thoracic wall invasive tumor revealed it to be a malignant peripheal nerve sheath tumor with rhabdomyoblastic differentiation (Triton tumor) with the positivity for the specific aforementioned markers and a proliferation index – Ki67 of 90%. Chemotherapy was the elective treatment which was followed for 1 month, the patient having a rapid downward evolution towards exitus, with a 33 month post-diagnosis survival. Conclusions: This case was presented due to the rarity and difficulty of the diagnosis, consisting of a rare subtype of malignant peripheral nerve sheath tumor for which there is no therapeutic consensus and with an un-favorable prognosis.
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spelling doaj.art-85efc9087e144200aefc7d4590b01fdb2022-12-21T20:44:18ZengMedia Med PublicisModern Medicine1223-04722360-24732021-03-012819397https://doi.org/10.31689/rmm.2021.28.1.93Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) TumorLaura REBEGEA0Elena NICULET1Mihaela CRAESCU2Dorel FIRESCU3Georgiana Bianca CONSTANTIN4Mihaela DUMITRU5Cristina SERBAN6Raul MIHAILOV7Mihaela LUNGU8Department of Radiotherapy, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaMedical Clinical Department, Faculty of Medicine and Pharmacy, „Dunarea de Jos” University, Galati, RomaniaDepartment of Radiotherapy, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaIInd Clinic of Surgery, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaIInd Clinic of Surgery, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaDepartment of Radiotherapy, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaIInd Clinic of Surgery, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaIInd Clinic of Surgery, „Sf. Apostol Andrei” Emergency Clinical Hospital, Galati, RomaniaMedical Clinical Department, Faculty of Medicine and Pharmacy, „Dunarea de Jos” University, Galati, RomaniaIntroduction: Immunohistochemistry is a widely used diagnostic technique in pathology, the antibodies used for unvailing a tissue’s origin being made up of proteins, more specifically aminoacids. Malignant peripheral nerve sheath tumors have a distinct immunohistochemistry profile, with great emphasis concerning those with rhabdomyoblastic differentiation being positive for S-100 protein, myogenin, vimentin, CD99, p63, GFAP, caldesmon, desmin, and p53 and Myo-D1 (the later two revealing the striated muscle differentiation). Materials and methods: A case study concerning a patient suffering from a moderately differentiated (G2) non-keratinizing squamous cell carcinoma of the lung highlighted the development of an undifferentiated sarcomatous proliferation on the left hemithorax, 7 years after the initial external radiotherapy treatment for the malignant lung tumor. Results: The immunohistochemistry analysis of the thoracic wall invasive tumor revealed it to be a malignant peripheal nerve sheath tumor with rhabdomyoblastic differentiation (Triton tumor) with the positivity for the specific aforementioned markers and a proliferation index – Ki67 of 90%. Chemotherapy was the elective treatment which was followed for 1 month, the patient having a rapid downward evolution towards exitus, with a 33 month post-diagnosis survival. Conclusions: This case was presented due to the rarity and difficulty of the diagnosis, consisting of a rare subtype of malignant peripheral nerve sheath tumor for which there is no therapeutic consensus and with an un-favorable prognosis.https://medicinamoderna.ro/wp-content/uploads/2021/03/Clinical-Histological-Immunohistochemical-12.pdfimmunohistochemistrymarker of proliferationtriton tumor
spellingShingle Laura REBEGEA
Elena NICULET
Mihaela CRAESCU
Dorel FIRESCU
Georgiana Bianca CONSTANTIN
Mihaela DUMITRU
Cristina SERBAN
Raul MIHAILOV
Mihaela LUNGU
Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
Modern Medicine
immunohistochemistry
marker of proliferation
triton tumor
title Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
title_full Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
title_fullStr Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
title_full_unstemmed Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
title_short Clinical, Histological, Immunohistochemical Aspects in a Rare Malignant Peripheral Nerve Sheath (Triton) Tumor
title_sort clinical histological immunohistochemical aspects in a rare malignant peripheral nerve sheath triton tumor
topic immunohistochemistry
marker of proliferation
triton tumor
url https://medicinamoderna.ro/wp-content/uploads/2021/03/Clinical-Histological-Immunohistochemical-12.pdf
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