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A Case of Multiple Micronodular Pneumocyte Hyperplasia of the Lung in a Man with Tuberous Sclerosis

Tuberculosis & Respiratory Diseases / Tuberculosis & Respiratory Diseases,
2008, v.64 no.5, pp.369-373









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Abstract

Tuberous sclerosis (TS) is an autosomal dominant disorder that is characterized by cutaneous lesions, seizures,mental retardation and hamartomas in various organs including the skin, kidney and brain. Pulmonary involvement is extremely rare, and occurs in approximately 0.1 to 1% of TS cases. Recent reports have indicated multiple micronodular pneumocyte hyperplasia (MMPH) as another rare form of pulmonary involvement of tuberous sclerosis. We report a case of a 35 year-old-male patient who had no pulmonary symptoms but showed multinodular pulmonary shadows on his chest CT scan. The patient was finally diagnosed with TS with MMPH of the lung. MMPH does not appear to have any malignant potential but the clinical significance of MMPH in TS patients is unknown.

keywords
Tuberous sclerosis, Multiple micronodular pneumocyte hyperplasia

Reference

1.

1. Sparagana SP, Roach ES. Tuberous sclerosis complex.Curr Opin Neurol 2000;13:115-9.

2.

2. Yagci C, Sahin-Akyar G, Akyar S. Multiple organ involvement in tuberous sclerosis. Eur J Radiol 1997;25:52-4.

3.

3. Costello LC, Hartman TE, Ryu JH. High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin Proc 2000;75:591-4.

4.

4. Maruyama H, Ohbayashi C, Hino O, Tsutsumi M,Konishi Y. Pathogenesis of multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis and association with tuberous sclerosis genes TSC1 and TSC2. Pathol Int2001;51:585-94.

5.

5. Maruyama H, Seyama K, Sobajima J, Kitamura K,Sobajima T, Fukuda T, et al. Multifocal micronodular pneumocyte hyperplasia and lymphangioleiomyomatosis in tuberous sclerosis with a TSC2 gene. Mod Pathol 2001;14:609-14.

6.

6. Popper HH, Juettner-Smolle FM, Pongratz MG. Micronodular hyperplasia of type II pneumocytes-a new lung lesion associated with tuberous sclerosis. Histopathology1991;18:347-54.

7.

7. Mo EK, Jung MP, Yoo CG, Kim YW, Han SK, Im JG,et al. Lymphangioleiomyomatosis in Korea. Tuberc Respir Dis 1993;40:519-31.

8.

8. Baik JM, Hong HK, Oh YB, Lee SM, Park MS, Yoo TK,et al. A case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis and renal angiomyolipoma.Tuberc Respir Dis 1997;44:1184-93.

9.

9. Shen A, Iseman MD, Waldron JA, King TE. Exacerbation of pulmonary lymphangioleiomyomatosis by exog enous estrogens. Chest 1987;91:782-5.

10.

10. Brentani MM, Carvalho CR, Saldiva PH, Pacheco MM,Oshima CT. Steroid receptors in pulmonary lymphangiomyomatosis.Chest 1984;85:96-9.

11.

11. Moss J, Avila NA, Barnes PM, Litzenberger RA, Bechtle J, Brooks PG, et al. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med 2001;164:669-71.

12.

12. Franz DN, Brody A, Meyer C, Leonard J, Chuck G,Dabora S, et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001;164:661-8.

13.

13. Fujitaka K, Isobe T, Oguri T, Yamasaki M, Miyazaki M,Kohno N, et al. A case of micronodular pneumocyte hyperplasia diagnosed through lung biopsy using thoracoscopy. Respiration 2002;69:277-9.

14.

14. Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, et al. Clear cell (“sugar”) tumor of the lung is a lesion strictly related to angiomyolipoma--the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC).Pathology 1994;26:230-6.

15.

15. Fujitaka K, Isobe T, Oguri T, Yamasaki M, Miyazaki M,Kohno N, et al. A case of micronodular pneumocyte hyperplasia diagnosed through lung biopsy using thoracoscopy. Respiration 2002;69:277-9.

Tuberculosis & Respiratory Diseases