Renal sclerosing peritubular nodules in a patient with neurofibromatosis type 2: a case report with immunohistochemical and electron microscopic studies

Summary Kidney lesions in patients with von Recklinghausen typically include blood vessels with smooth muscle proliferation resulting in hypertension, and perirenal nerve sheath tumors. In the English language literature, there is only 1 report published more than 25 years ago that described a previ...

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Bibliographic Details
Published in:Human pathology 2009-11, Vol.40 (11), p.1650-1654
Main Authors: Gökden, Neriman, MD, El Jamal, Siraj, MD, Gökden, Murat, MD, Lamps, Laura W., MD
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Collagen
Fatal Outcome
Hospice care
Humans
Immunohistochemistry
Investigative techniques, diagnostic techniques (general aspects)
Kidney
Kidney Diseases - etiology
Kidney Diseases - pathology
Kidney Tubules - pathology
Male
Medical sciences
Microscopy, Electron, Transmission
Neurofibromatosis
Neurofibromatosis 2 - complications
Neurofibromatosis 2 - pathology
Neurology
Pathology
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
Peritubular nodules
Proteins
Smooth muscle
Tumors of the nervous system. Phacomatoses
Von Recklinghausen disease
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Summary:Summary Kidney lesions in patients with von Recklinghausen typically include blood vessels with smooth muscle proliferation resulting in hypertension, and perirenal nerve sheath tumors. In the English language literature, there is only 1 report published more than 25 years ago that described a previously unrecognized renal parenchymal lesion, termed sclerosing peritubular nodules, in 3 members of a family affected by von Recklinghausen disease. These nodules were examined by light microscopy, histochemistry, and electron microscopy; they proved to be derived from interstitial cells, and the proliferating cells showed myofibroblastic differentiation by electron microscopy. We report a case of a 22-year-old man with hereditary neurofibromatosis type 2 who died with complications of the disease. He underwent autopsy, and his kidneys showed this unique lesion. We examined these peritubular proliferations with immunohistochemistry, histochemistry, and electron microscopy and compared our findings with the previous report in the literature.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2009.04.014