Tuberc Respir Dis.  2015 Oct;78(4):463-468. 10.4046/trd.2015.78.4.463.

Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

Affiliations
  • 1Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea. sanghakim@yonsei.ac.kr
  • 2Department of Radiology, Yonsei University Wonju College of Medicine, Wonju, Korea.
  • 3Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea.
  • 4Department of Thoracic and Cardiovascular Surgery, Yonsei University Wonju College of Medicine, Wonju, Korea.

Abstract

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

Keyword

Histiocytosis, Langerhans-Cell; Cystic Disease of Lung; Diabetes Insipidus; Diabetes Mellitus; Smoking Cessation

MeSH Terms

Adult*
Biopsy
Cough
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Diabetes Mellitus*
Diabetes Mellitus, Type 2
Dyspnea
Follow-Up Studies
Histiocytosis, Langerhans-Cell*
Humans
Lung
Lung Diseases
Magnetic Resonance Imaging
Male*
Polydipsia
Smoke
Smoking
Smoking Cessation
Thoracic Surgery, Video-Assisted
Water Deprivation
Smoke

Figure

  • Figure 1 Magnetic resonance imaging of the hypothalamic-pituitary system. The hyperintense signal of the posterior pituitary on the T1-weighted image could not be detected. Lack of this bright spot (arrow) is typical of central diabetes insipidus.

  • Figure 2 Computed tomographic scans of the chest. (A) Multiple irregular cysts and centrilobular nodules can be seen in both lung fields, with sparing of the costophrenic angles. (B) Follow-up scan at 1-year after the patient stopped smoking. The size and thickness of the irregular cysts are reduced, and both lungs show a decrease in the number of centrilobular nodules.

  • Figure 3 Langerhans cell histiocytosis. (A) The cut surface of wedge-resected lung shows numerous cystic spaces with whitish gray stellate fibrous scars. (B) Multiple cystic spaces are evident, with diffuse thickening, cellular infiltration, and fibrous tissue (H&E stain, ×12.5). (C) The cytoplasm of the infiltrated cells is pale and eosinophilic, and the nuclei are grooved or infolded (H&E stain, ×400). (D) Immunohistochemical staining of the proliferating cells is diffuse and strongly positive for CD1a (×400).


Reference

1. Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans'-cell histiocytosis. N Engl J Med. 2000; 342:1969–1978.
2. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med. 2002; 346:484–490.
3. Kim C, Jeong SH, Shim JJ, Cha SI, Son C, Chung MP, et al. Clinical features of pulmonary Langerhans cell histiocytosis in Korea. Tuberc Respir Dis. 2009; 66:98–103.
4. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer. 1999; 85:2278–2290.
5. Girschikofsky M, Arico M, Castillo D, Chu A, Doberauer C, Fichter J, et al. Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis. 2013; 8:72.
6. Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans' cell histiocytosis: radiologic resolution following smoking cessation. Chest. 1999; 115:1452–1455.
7. Alexandraki KI, Makras P, Protogerou AD, Dimitriou K, Stathopoulou A, Papadogias DS, et al. Cardiovascular risk factors in adult patients with multisystem Langerhans-cell histiocytosis: evidence of glucose metabolism abnormalities. QJM. 2008; 101:31–40.
8. Thai AC, Sng I, Suri R, Cheah JS. Disseminated histiocytosis X with diabetes insipidus and diabetes mellitus in an adult female (histiocytosis with DI and DM). Ann Acad Med Singapore. 1988; 17:294–298.
9. Rowntree LG, Poppiti RJ. Diabetes inspidius, diabetes mellitus, and insulin resistance with histiocytosis. J Am Med Assoc. 1954; 156:310–312.
10. Hong ES, Ohn JH, Kim JH, Hwang-Bo YK, Kim JJ, Kwon JH, et al. Clinical characteristics of Langerhans cell histiocytosis with hypothalamo-pituitary involvement. Endocrinol Metab. 2011; 26:38–43.
11. Choi JE, Lee HR, Ohn JH, Moon MK, Park J, Lee SJ, et al. Adult multisystem langerhans cell histiocytosis presenting with central diabetes insipidus successfully treated with chemotherapy. Endocrinol Metab. 2014; 29:394–399.
12. Makras P, Samara C, Antoniou M, Zetos A, Papadogias D, Nikolakopoulou Z, et al. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH). Neuroradiology. 2006; 48:37–44.
13. Grois N, Prayer D, Prosch H, Minkov M, Potschger U, Gadner H. Course and clinical impact of magnetic resonance imaging findings in diabetes insipidus associated with Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004; 43:59–65.
14. Arico M, Girschikofsky M, Genereau T, Klersy C, McClain K, Grois N, et al. Langerhans cell histiocytosis in adults: report the International Registry of the Histiocyte Society. Eur J Cancer. 2003; 39:2341–2348.
15. Moore AD, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, et al. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology. 1989; 172:249–254.
16. Hansen NJ, Hankins JH. Pulmonary langerhans cell histiocytosis: PET/CT for initial workup and treatment response evaluation. Clin Nucl Med. 2015; 40:153–155.
17. Lee HJ, Ahn BC, Lee SW, Lee J. The usefulness of F-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Langerhans cell histiocytosis. Ann Nucl Med. 2012; 26:730–737.
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