Tuberous Sclerosis Complex Complicated by Pulmonary Multinodular Shadows

Kamiya, Hiroyuki; Shinoda, Kinya; Kobayashi, Nobuyuki; Kudo, Koichiro; Nomura, Tomokiyo; Morita, Takatomo; Fujii, Takeshi

doi:10.2169/internalmedicine.45.1136

Cited by 7 publications

(5 citation statements)

References 17 publications

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“…MMPH represents benign overexpression of type II pneumocytes along with alveolar septa that reveal fibrous thickening, increase in elastic fibers, and aggregates of alveolar macrophages. MMPH has an unclear prognosis and is very unlikely to have malignant progression, so management is conservative [ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex

Ijaz

Rafiq

Venkatram

et al. 2015

Case Reports in Critical Care

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Tuberous sclerosis complex (TSC) is an autosomal dominant, variably expressed multisystem disease. The predominant pulmonary features of TSC are identical to those of lymphangioleiomyomatosis (LAM). Pneumothorax, multifocal micronodular pneumocyte hyperplasia, and chylothorax are rare complications of TSC. We report a young male with pneumothorax, lung nodules, and chylous effusion who developed empyema thoracis after esophageal rupture. Hospital course was complicated by respiratory failure. Family opted to transfer to hospice care. Chylothorax is a rare complication of TSC with few scattered reports mostly in female patients. Patients with TSC are usually managed by multispecialists and it is important to be aware of the rare pulmonary manifestations of this disease. A male patient with TSC having lung nodules presenting with chylothorax and empyema thoracis from Boerhaave syndrome makes our case unique.

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Section: Discussionmentioning

confidence: 99%

Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex

Ijaz

Rafiq

Venkatram

et al. 2015

Case Reports in Critical Care

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“…Chylothorax is an uncommon but vexing clinical disorder, and may be a life-threatening condition if left untreated. [3–5]…”

Section: Discussionmentioning

confidence: 99%

VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex

et al. 2013

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Lymphangioleiomyomatosis with tuberous sclerosis complex is a rare disease. One of the most frequent complications of lymphangioleiomyomatosis is pleural effusion (chylothorax) wich can be treated with the use of VATS. Authors report a case of pulmonary lymphangioleiomyomatosis in a 56-year-old female patient with tuberous sclerosis complex with an 8-week history of recurrent chylothorax, dyspnea and debilitating weakness. By CT scan a flat tissue proliferation was seen in the site of the thoracic duct and it was supposed to be the reason for the pleural effusion. A VATS resection of this laesion and ligation of the thoracic duct was performed successfully. Chylothorax is often associated with pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis combined with tuberous sclerosis complex is extremely rare. In case of chylothorax VATS treatment is successful and may be the first choice.

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“…in 1991 2 . Although there have been sporadic case reports on MMPH with TSC in Japan, 3–16 there is no clinical or pathological data from a series of patients with MMPH of the lung, associated with TSC. In this study, we analysed the clinical and pathological findings in Japanese patients, diagnosed by surgical procedures as having MMPH with TSC.…”

Section: Introductionmentioning

confidence: 99%

“…1 Multifocal micronodular pneumocyte hyperplasia (MMPH) is extremely rare and is another pulmonary manifestation of TSC, which was described by Popper et al in 1991. 2 Although there have been sporadic case reports on MMPH with TSC in Japan, [3][4][5][6][7][8][9][10][11][12][13][14][15][16] there is no clinical or pathological data from a series of patients with MMPH of the lung, associated with TSC. In this study, we analysed the clinical and pathological findings in Japanese patients, diagnosed by surgical procedures as having MMPH with TSC.…”

Section: Introductionmentioning

confidence: 99%

Clinicopathological analysis of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis in Japan

Kobashi¹,

Sugiu²,

Mouri³

et al. 2008

Respirology

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(80%) were women and the mean age was 37 years. Three patients had a family history of TSC. The radiological findings were small multiple nodular shadows with ground-glass opacity randomly distributed in the bilateral whole-lung fields in most patients. Differentiation from multiple atypical adenomatous hyperplasia or metastatic lung cancer was necessary in most patients. In 11 patients, the diagnostic method used to identify pulmonary lesions of MMPH with TSC was VAT. Among the complications of MMPH with TSC, lymphangioleiomyomatosis was recognized in 53% of patients. The prognosis was comparatively good because only one patient died of respiratory failure. The histological findings were papillary or tubular proliferation of type II pneumocytes without nuclear atypia lining the thickened alveolar septa and lymphocyte infiltration. Immunohistochemical staining for cytokeratin, and surfactant proteins A and B was positive in alveolar lining cells of all MMPH lesions. However, staining for HMB-45, a-smooth muscle actin, p53, carcinoembryonic antigen and hormonal receptor was negative in most patients. Conclusions: Surgical investigation and immunohistochemical staining for pathological markers are useful for diagnosing MMPH when the radiological findings indicate multiple micronodules with ground-glass opacity in patients with TSC.

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Tuberous Sclerosis Complex Complicated by Pulmonary Multinodular Shadows

Abstract: Abstract

Cited by 7 publications

References 17 publications

Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex

Boerhaave Syndrome, Pneumothorax, and Chylothorax in a Critically Ill Patient with Tuberous Sclerosis Complex

VATS therapy of chylothorax caused by leiomyomatosis complicated with tuberous sclerosis complex

Clinicopathological analysis of multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis in Japan

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