Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis

Ristagno, Ross L.; Biddinger, Paul; Pina, Elsira M.; Meyer, Catalina M.

doi:10.2214/ajr.184.3_supplement.01840s37

Cited by 34 publications

(25 citation statements)

References 13 publications

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“…Pulmonary manifestations are estimated to occur in approximately 1%-2.3% of TS patients, but recent reports indicate that pulmonary LAM can be found radiologically in 26%-39% of female patients with TS (38). MMPH is a rare disorder that has been described in few literature reports.…”

Section: Pulmonary and Thoracic Involvementmentioning

confidence: 99%

Pictorial Review of Tuberous Sclerosis in Various Organs

Umeoka¹,

Koyama²,

Miki³

et al. 2008

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Tuberous sclerosis is a rare autosomal dominant neurocutaneous syndrome characterized by the presence of benign congenital tumors in multiple organs. The diagnosis is usually established on the basis of diagnostic criteria applied to physical or radiologic findings. Because the classical triad of epilepsy, mental retardation, and adenoma sebaceum is uncommonly seen at clinical examination, radiologic examinations can play an important role in the diagnosis of tuberous sclerosis and in treatment. Cardiac rhabdomyoma, renal angiomyolipoma, and neurologic involvement encompassing cortical or subependymal tubers and white matter abnormalities are the common radiologic findings. Detection of these entities can be strong evidence for suspecting tuberous sclerosis. The presence of pulmonary lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, or multiple renal cysts also raises suspicion of tuberous sclerosis. Moreover, tuberous sclerosis can involve bone, liver, and the alimentary tract. The clinical course and patient prognosis depend on the sites of manifestations. Familiarity with the clinical and radiologic findings in various organs is crucial in diagnosis and treatment.

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Section: Pulmonary and Thoracic Involvementmentioning

confidence: 99%

Pictorial Review of Tuberous Sclerosis in Various Organs

Umeoka¹,

Koyama²,

Miki³

et al. 2008

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189

174

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“…MMPH is reported in the literature with multiple small nodules in the bilateral lung fi elds in TS patients. Since our patient did not have any granulomatous or metastatic disease, diagnosis of MMPH was considered [5,10,11]. It is characterized by multicentric, well-demarcated nodular proliferation of type II pneumocytes along alveolar septa [11].…”

Section: Discussionmentioning

confidence: 98%

“…Th e most common renal lesions in the patients with TS are the angiomyolipomas; these are rarely seen benign neoplasms which are composed of varying amounts of mature adipose tissue, smooth muscles, and blood vessels [3,4]. MMPH is a hamartomatous process of the lung that exhibits multiple tiny pulmonary nodules [5]. In this article, we are reporting the case of a female patient with massive bilateral renal angiomyolipomas (AML), multifocal micronodular pneumocyte hyperplasia (MMPH), and tuberous sclerosis (TS).…”

Section: Introductionmentioning

confidence: 99%

Massive bilateral renal angiomyolipomatosis and multifocal micronodular pneumocytes hyperplasia associated with tuberous sclerosis: A case report

İncedayı

Sönmez

Başekim

2011

Wien Klin Wochenschr

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Tuberous sclerosis (TS) is an autosomal-dominant disorder characterized by a variety of hamartomatous lesions in various organs. Various organ involvements can be detected with radiology: neurologic involvement (cortical tubers, subependymal nodules and white matter abnormalities), renal involvement (angiomyolipoma) and pulmonary involvement (pulmonary lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia). Here, we are reporting a case of tuberous sclerosis with multiple organ involvement and massive bilateral AMLs in a 49-year-old female patient.

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“…MMPH is a hamartomatous process of the lungs, which manifests at imaging with diffusely scattered, randomly distributed nodules measuring 1 to 8 mm in diameter. 13,14 Renal angiomyolipomas (AMLs) are the most common extrathoracic manifestation of LAM and are seen more commonly in TSC-LAM. 15 AMLs characteristically appear as partially fatattenuating lesions on CT (see Fig.…”

Section: Cystic Lung Diseasementioning

confidence: 99%