Abstract
Differentiation among various non-Langerhans cell histiocytoses granulomatous in adults is often difficult. Patients, moreover, may not have endocrinologic abnormalities. A 53-yr-old patient was admitted owing to central diabetes insipidus and partial hypopituitarism. Magnetic resonance imaging revealed a space-occupying lesion near the hypophyseal stalk, along with diffuse signal uptake in the cerebellar region. Laboratory chemistry showed monoclonal gammopathy of IgGκ, and hormone tests disclosed insufficiency in the gonadotropic and somatotropic axes. The clinical picture was marked by multiple cutaneous xanthogranulomas, ataxic gait with blurred speech, compatible with pseudobulbar pontocerebellar symptomatology. Stereotactic pituitary biopsy was histologically classified as nonspecific granulomatous disease. Supplemental biopsies taken from the cutaneous periorbital xanthogranulomas were histologically and immunohistochemically consistent with “non-Langerhans cell histiocytosis.” Systemic cortisone treatment as well as local radiotherapy to the pituitary lesion with a total of 18.0 gy had no impact on the progression of disease-growing tumor and progressing neurologic symptoms. Systemic granulomatoses cannot always be classified according to specific defined diseases. Differential diagnosis in the current patient should include the possibility of Erdheim-Chester disease, necrobiotic xanthogranuloma, and adult disseminated xanthoma.
Similar content being viewed by others
References
Tritos NA, Weinrib S, Kaye TB. Endocrine manifestations of Erdheim-Chester disease (a distinct form of histiocytosis). J Int Med 244:529–535, 1998.
Miller RL, Sheeler IR, Bauer TW, Bukowski RM. Erdheim-Chester disease: case report and review of literature. Am J Med 80:1239–1246, 1986.
Capparos-Lefebvre D, Pruvo JP, Remy M, Wallaert B, Petit H. Neuroradiologic aspects of Erdheim-Chester disease. AJNR 16:735–740, 1995.
Foldert RD, Price DL Jr, Schwartz M, Black PM, De Girolami U. Xantomatous hypophysitis. Am J Surg Pathol (22)6:736–741, 1998.
Veysier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. Erdheim-Chester disease: clinical and radiological characteristics of 59 cases. Medicine 75:157–169, 1996.
Johnston KA, Grimwood RE, Meffert JJ, Deering KC. Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. Cutis 59(6):333–336, 1997.
Caputo R, Veraldi S, Grimalt R, et al. The various clinical patterns of xanthoma disseminatum. Dermatology 190:19–24, 1995.
Finelli LG, Ratz JL. Plasmapheresis: a treatment modality for necrobiotic xanthogranuloma. J Am Acad Dermatol 17:351–354, 1987.
Pfeiff B, Straube R, Pullmann H, Kingreen H. Erfahrungen mit therapeutischer plasmapherese. H+G 70(1):48–52, 1995.
Traupe H, Hamm H. Pädiatrische Dermatologie. Springer Verlag, Berlin, 1999.
Zelger BWH, Sidoroff A, Orchard G, Cerio R. Non-Langerhans-cell histiocytoses: a new unifying concept. Am J Dermatopathol 18(5):490–504, 1996.
Wright RA, Hermann RC, Parisi JE. Neurological manifestations of Erdheim-Chester disease. J Neurol Neurosurg Psychiatry 66(1):72–75, 1999.
Finan MC, Winkelmann RK. Necrobiotic xanthogranuloma with paraproteinemia. Medicine 65:376–385, 1986.
Finan MC, Winkelmann RK. Histopathology of necrobiotic xanthogranuloma. J Cutan Pathol 14:92–99, 1987.
Char DH, Le Boit Phe, Ljung BME, Wara W. Radiation therapy for ocular necrobiotic xanthogranuloma. Arch Ophthalmol 105:174, 175, 1987.
Kyle RA. Monoclonal gammopathy of undetermined significance (MGUS). Baillieres Clin Haematol 8(4):761–781, 1995.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mahnel, R., Tan, K.H., Fahlbusch, R. et al. Problems in differential diagnosis of non-langerhans cell histiocytosis with pituitary involvement: Case report and review of literature. Endocr Pathol 13, 361–368 (2002). https://doi.org/10.1385/EP:13:4:361
Issue Date:
DOI: https://doi.org/10.1385/EP:13:4:361