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Presented by Ralph Hruban, M.D. and prepared by Zarir E. Karanjawala, M.D., Ph.D.
Case 1: This young adult with neurofibromatosis type 1 was found to have a large soft tissue mass in his leg.
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1. Question
Week 345: Case 1
This young adult with neurofibromatosis type 1 was found to have a large soft tissue mass in his leg.images/hruban032408_1A.jpg
images/hruban032408_1b.jpg
images/hruban032408_1c.jpgCorrect
Answer: Pigmented neurofibroma
Histology: This spindle cell neoplasm is composed of long thin cells with serpentine nuclei. The cells are embedded in a collagenous stroma. The nuclei are uniform and mitotic figures are not appreciated. In addition, numerous cells contain a brown pigment.
Discussion: A number of neoplasms can be pigmented. In addition to the classical melanocytic neoplasms, pigmented neoplasms include the Bednár Tumor (Pigmented dermatofibrosarcoma protuberans), pigmented endocrine neoplasms (carcinoid, adrenal and pancreatic endocrine neoplasms), and the pigmented neurofibroma. Pigmentation in such a wide variety of neoplasms should not be surprising considering the large number of structures in the body to which the neural crest contributes. The neural crest contributes to the ganglia (sympathetic and parasympathetic), glia, Schwann cells, melanocytes, adrenal medulla, c-cells of the thyroid, odontoblasts, and eye (cornea and sclera).
Pigmented neurofibromas are entirely benign and are composed of short spindle cells admixed with melanin-laden cells. Immunohistochemical labeling will reveal that the spindle cells express S-100 protein, while the melanin-laden cells will label with HMB-45.
Reference(s):
– Inaba et al., Pigmented neurofibroma: report of two cases and literature review. Pathol Int. 2001 Jul;51(7):565-9.Incorrect
Answer: Pigmented neurofibroma
Histology: This spindle cell neoplasm is composed of long thin cells with serpentine nuclei. The cells are embedded in a collagenous stroma. The nuclei are uniform and mitotic figures are not appreciated. In addition, numerous cells contain a brown pigment.
Discussion: A number of neoplasms can be pigmented. In addition to the classical melanocytic neoplasms, pigmented neoplasms include the Bednár Tumor (Pigmented dermatofibrosarcoma protuberans), pigmented endocrine neoplasms (carcinoid, adrenal and pancreatic endocrine neoplasms), and the pigmented neurofibroma. Pigmentation in such a wide variety of neoplasms should not be surprising considering the large number of structures in the body to which the neural crest contributes. The neural crest contributes to the ganglia (sympathetic and parasympathetic), glia, Schwann cells, melanocytes, adrenal medulla, c-cells of the thyroid, odontoblasts, and eye (cornea and sclera).
Pigmented neurofibromas are entirely benign and are composed of short spindle cells admixed with melanin-laden cells. Immunohistochemical labeling will reveal that the spindle cells express S-100 protein, while the melanin-laden cells will label with HMB-45.
Reference(s):
– Inaba et al., Pigmented neurofibroma: report of two cases and literature review. Pathol Int. 2001 Jul;51(7):565-9.
