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Presented by Ashley Cimino-Mathews, MD and prepared by Armen Khararjian, MD.
This case talks about:
A 55 year-old female with a scalp mass.
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Question 1 of 1
1. Question
Week 620: Case 3
A 55 year-old female with a scalp mass.
Correct
Answer: Malignant Peripheral Nerve Sheath Tumor
Histology: The resection of this scalp mass reveals highly atypical epithelioid cells with prominent nucleoli, nuclear hyperchromasia and abundant mitotic figures. There are regions of geographic necrosis and a low-power impression of “zonation” to the lesion, with some hypercellular and hypocellular regions. Adjacent to the malignant lesion is a background of bland spindled cells with wavy nuclei, delicate chromatin, and indistinct cellular borders. Numerous mast cells are present. The stromal collagen has a wavy, pink appearance resembling “shredded carrots.” These features are in keeping with a background of diffuse neurofibroma.
Discussion: This lesion is a malignant peripheral nerve sheath tumor (MPNST) arising in a pre-existing diffuse neurofibroma. This patient does not have a history of neurofibromatosis, but MPNST may arise de novo, in association with a sporadic neurofibroma, or in association with a neurofibroma as a part of neurofibromatosis. Syndromic neurofibromas have a greater likelihood of malignant transformation than sporadic neurofibromas. Diffuse neurofibromas, as seen here, are infiltrative, non-encapsulated lesions which entrap adnexal structures, skeletal muscle or adipose tissue, and they can arise sporadically or syndromically. Conventional MPST are cellular sarcomas comprised of spindled cells with wavy nuclei, tapering ends, and fascicular growth. The cells characteristically condense around hyalizined blood vessels within the neoplasm and may show geographic necrosis similar to that seen in glioblastoma. There are several morphologic varieties of MPNST, including epithelioid MPNST and Triton tumor (which skeletal muscle differentiation morphologically or immunophenotypically). The MPNST in this case displays abundant epithelioid features, with plump rather than spindled cells. The differential diagnosis of MPNST includes other sarcomas such as undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma, MFH), pleomorphic liposarcoma, leiomyosarcoma, fibrosarcoma, synovial sarcoma, as well as sarcomatoid carcinoma and melanoma. Conventional MPNST is only focally immunoreactive for S-100, but epithelioid MPNST can be diffusely positive. The presence of the associated neurofibroma supports classification as an MPNST.
References:
1. Cimino-Mathews A. Peripheral nerve sheath tumors. In Surgical Pathology Clinics: Current Concepts in Soft Tissue Pathology. Guest Editor, EA Montgomery. Consulting Editor, J Goldblum. 2011 (4); 761-782.
2. Evans DG, Baser ME, McGaughran J, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002 May;39(5):311-4.
3. Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: A twenty first century perspective. Lancet Neurol 2007;6(4):340-351.
4. McClatchey AI. Neurofibromatosis. Annu Rev Pathol 2007;2:191-216.
5. Wanebo JE, Malik JM, VandenBerg SR, et al. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer 1993;71(4):1247-1253.Incorrect
Answer: Malignant Peripheral Nerve Sheath Tumor
Histology: The resection of this scalp mass reveals highly atypical epithelioid cells with prominent nucleoli, nuclear hyperchromasia and abundant mitotic figures. There are regions of geographic necrosis and a low-power impression of “zonation” to the lesion, with some hypercellular and hypocellular regions. Adjacent to the malignant lesion is a background of bland spindled cells with wavy nuclei, delicate chromatin, and indistinct cellular borders. Numerous mast cells are present. The stromal collagen has a wavy, pink appearance resembling “shredded carrots.” These features are in keeping with a background of diffuse neurofibroma.
Discussion: This lesion is a malignant peripheral nerve sheath tumor (MPNST) arising in a pre-existing diffuse neurofibroma. This patient does not have a history of neurofibromatosis, but MPNST may arise de novo, in association with a sporadic neurofibroma, or in association with a neurofibroma as a part of neurofibromatosis. Syndromic neurofibromas have a greater likelihood of malignant transformation than sporadic neurofibromas. Diffuse neurofibromas, as seen here, are infiltrative, non-encapsulated lesions which entrap adnexal structures, skeletal muscle or adipose tissue, and they can arise sporadically or syndromically. Conventional MPST are cellular sarcomas comprised of spindled cells with wavy nuclei, tapering ends, and fascicular growth. The cells characteristically condense around hyalizined blood vessels within the neoplasm and may show geographic necrosis similar to that seen in glioblastoma. There are several morphologic varieties of MPNST, including epithelioid MPNST and Triton tumor (which skeletal muscle differentiation morphologically or immunophenotypically). The MPNST in this case displays abundant epithelioid features, with plump rather than spindled cells. The differential diagnosis of MPNST includes other sarcomas such as undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma, MFH), pleomorphic liposarcoma, leiomyosarcoma, fibrosarcoma, synovial sarcoma, as well as sarcomatoid carcinoma and melanoma. Conventional MPNST is only focally immunoreactive for S-100, but epithelioid MPNST can be diffusely positive. The presence of the associated neurofibroma supports classification as an MPNST.
References:
1. Cimino-Mathews A. Peripheral nerve sheath tumors. In Surgical Pathology Clinics: Current Concepts in Soft Tissue Pathology. Guest Editor, EA Montgomery. Consulting Editor, J Goldblum. 2011 (4); 761-782.
2. Evans DG, Baser ME, McGaughran J, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002 May;39(5):311-4.
3. Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: A twenty first century perspective. Lancet Neurol 2007;6(4):340-351.
4. McClatchey AI. Neurofibromatosis. Annu Rev Pathol 2007;2:191-216.
5. Wanebo JE, Malik JM, VandenBerg SR, et al. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 28 cases. Cancer 1993;71(4):1247-1253.
