Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases

Mentzel, Thomas; Katenkamp, D.

doi:10.1046/j.1365-2559.1999.00714.x

Cited by 72 publications

(41 citation statements)

References 21 publications

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“…Several spindle cell neoplasms may grow inside the nerve trunks, including perineurioma 31,32 and peripheral nerve sheath tumor. [33][34][35] SS with a biphasic component may be easier to diagnose, but the monophasic presentation becomes a diagnosis by exclusion, and needs more sophisticated techniques, particularly molecular biology, to reach a proper diagnosis with certainty. The spindle cell tumor most often encountered in nerve trunk is a peripheral nerve sheath tumor (PNST), including both the benign neurofibroma or schwannoma and the malignant peripheral nerve sheath tumor (malignant schwannoma).…”

Section: Discussionmentioning

confidence: 99%

Intraneural synovial sarcoma: two cases

et al. 2004

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We report two cases of intraneural synovial sarcoma. The first patient is a 46-year-old female who presented for several months with soft-tissue mass in the right infra-auricular region. The second patient is a 11-year-old girl who fell and then presented with pain in the area innervated by the right C7 spinal root and a nodule identified in the nerve root foramina. Both lesions were of small size and presented with features of synovial sarcoma. A biphasic variant was found in case 1 and a monophasic variant was present in case 2. Immunohistochemical studies were performed to confirm the diagnosis, excluding the main differential diagnoses, namely schwannoma and malignant peripheral nerve sheath tumor. Ultrastructural study was performed in case 2 allowing exclusion of other possible diagnoses. Molecular studies were performed on paraffin-embedded tissue in both cases and revealed the known characteristic t(X;18)(SYT-SSX) translocation.

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Section: Discussionmentioning

confidence: 99%

Intraneural synovial sarcoma: two cases

et al. 2004

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“…An angiosarcoma rarely develops in the setting of neurofibroma, neurofibromatosis type 1 or 2 or malignant peripheral nerve sheath tumour (MPNST) [10]. Our patient had diffuse neurofibroma, but not neurofibromatosis type 1 or 2.…”

Section: Discussionmentioning

confidence: 78%

Angiosarcoma With Malignant Peripheral Nerve Sheath TumourDeveloping in a Patient With Klippel–Trénaunay–Weber Syndrome

Ploegmakers

Pruszczyński

Rooy

et al. 2000

Sarcoma

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Purpose: We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. Patient: We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing his fibula. Since birth he was known as having the uncommon syndrome of Klippel-Trénaunay-Weber of his right lower leg. Methods: Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases. Results: In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months postoperatively. Discussion: This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trénaunay-Weber syndrome. A case never described before in literature and probably, as in our case, difficult to diagnose at first biopsy.

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“…In most cases of neurilemmoma with malignant transformation, the malignant component revealed predominantly or purely epithelioid differentiation (8,9). In the minor cases, the malignant component showed small cells with neuroepithelial differentiation (8) and angiosarcoma (9,19). Woodruff et al (8) reviewed nine cases of MPNSTs arising in benign neurilemmomas and described clinical and pathologic differences between MPNSTs arising in neurilemmomas and conventional MPNSTs.…”

Section: Discussionmentioning

confidence: 99%

Malignant Transformation of Intrathoracic Ancient Neurilemmoma in a Patient without Von Recklinghausen's Disease

et al. 2003

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Malignant transformation of a neurilemmoma is an exceedingly rare event. We describe a case of intrathoracic ancient neurilemmoma undergoing a malignant change in a 39-yr-old man. The patient presented with right flank and chest pain for several months. Plain radiography and CT scan of the chest showed a soft tissue mass lesion at the extrapleural space with erosion of surrounding ribs at the right basal lung area. The excised mass was encapsulated and measured 4.5 x 3.5 x 2.3 cm. The cut surface showed grayish-white and glistening with a focal cystic change and hemorrhage. Necrosis was not seen. Histologically, the tumor showed the features of classic neurilemmoma composed of the Antoni type A and B areas with perivascular hyalinization. In addition, obviously histo-logically malignant foci manifested by presence of markedly increased cellularity with fascicular arrangement, active mitotic activity, hyperchromasia, and gradual loss of original neurilemmomatous feature were noted.

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Intraneural angiosarcoma and angiosarcoma arising in benign and malignant peripheral nerve sheath tumours: clinicopathological and immunohistochemical analysis of four cases

Abstract: This study confirms the rare association of angiosarcoma arising in peripheral nerves, as well as in benign and malignant peripheral nerve sheath tumours.

Cited by 72 publications

References 21 publications

Intraneural synovial sarcoma: two cases

Intraneural synovial sarcoma: two cases

Angiosarcoma With Malignant Peripheral Nerve Sheath TumourDeveloping in a Patient With Klippel–Trénaunay–Weber Syndrome

Malignant Transformation of Intrathoracic Ancient Neurilemmoma in a Patient without Von Recklinghausen's Disease

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