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Profuse coarse pulmonary nodules in a patient with lymphangioleiomyomatosis: thirty-three years of follow-up.

Rasmussen DB, Shaker SB, Seersholm N, Colella S, Clementsen PF - Eur Clin Respir J (2014)

Bottom Line: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destruction of the lungs.We present an unusual radiological presentation of lymphangioleiomyomatosis in a patient followed for 33 years with profuse coarse lung nodules in addition to the classical cystic lesions.We believe that this report might support the case for considering LAM a low-malignant neoplasm.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Gentofte University Hospital, Hellerup, Denmark.

ABSTRACT
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destruction of the lungs. We present an unusual radiological presentation of lymphangioleiomyomatosis in a patient followed for 33 years with profuse coarse lung nodules in addition to the classical cystic lesions. We believe that this report might support the case for considering LAM a low-malignant neoplasm.

No MeSH data available.


Related in: MedlinePlus

(a) HRCT showing nodules (white arrows) and cysts (black arrows). (b) Transbronchial biopsy of the lung (estrogen receptor immunohistochemical stain).
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Figure 0001: (a) HRCT showing nodules (white arrows) and cysts (black arrows). (b) Transbronchial biopsy of the lung (estrogen receptor immunohistochemical stain).

Mentions: In 2010 HRCT was obtained for the first time, showing a few large thin-walled cysts and multiple coarse nodular changes distributed evenly in both lungs. The nodules varied in size from 3 to 2 cm. In 2012 a new HRCT showed slight increase in the size and number of nodules (Fig. 1a). Transbronchial biopsies showed a nodule with abnormal proliferation of spindle-shaped cells in fascicular streaks (Fig. 1b). These cells had elongated, hyperchromatic uniform nuclei. This area extended into the adjacent alveolar tissue without a distinct boundary. In the center of this area, there was a small cystic lesion. There was no evidence of interstitial inflammation, fibrosis, vasculitis, or granuloma formation. The alveolar septae were thickened in some areas. Immunohistochemical studies showed positive reaction of the spindle-shaped cells to estrogen, progesterone, desmin, vimentin, and alpha-smooth muscle actin. Human Melanoma Black 45 (HMB45), S-100, and MelanA were negative. It was concluded that the lesions were characteristic of LAM, and the diagnosis was confirmed.


Profuse coarse pulmonary nodules in a patient with lymphangioleiomyomatosis: thirty-three years of follow-up.

Rasmussen DB, Shaker SB, Seersholm N, Colella S, Clementsen PF - Eur Clin Respir J (2014)

(a) HRCT showing nodules (white arrows) and cysts (black arrows). (b) Transbronchial biopsy of the lung (estrogen receptor immunohistochemical stain).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4629714&req=5

Figure 0001: (a) HRCT showing nodules (white arrows) and cysts (black arrows). (b) Transbronchial biopsy of the lung (estrogen receptor immunohistochemical stain).
Mentions: In 2010 HRCT was obtained for the first time, showing a few large thin-walled cysts and multiple coarse nodular changes distributed evenly in both lungs. The nodules varied in size from 3 to 2 cm. In 2012 a new HRCT showed slight increase in the size and number of nodules (Fig. 1a). Transbronchial biopsies showed a nodule with abnormal proliferation of spindle-shaped cells in fascicular streaks (Fig. 1b). These cells had elongated, hyperchromatic uniform nuclei. This area extended into the adjacent alveolar tissue without a distinct boundary. In the center of this area, there was a small cystic lesion. There was no evidence of interstitial inflammation, fibrosis, vasculitis, or granuloma formation. The alveolar septae were thickened in some areas. Immunohistochemical studies showed positive reaction of the spindle-shaped cells to estrogen, progesterone, desmin, vimentin, and alpha-smooth muscle actin. Human Melanoma Black 45 (HMB45), S-100, and MelanA were negative. It was concluded that the lesions were characteristic of LAM, and the diagnosis was confirmed.

Bottom Line: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destruction of the lungs.We present an unusual radiological presentation of lymphangioleiomyomatosis in a patient followed for 33 years with profuse coarse lung nodules in addition to the classical cystic lesions.We believe that this report might support the case for considering LAM a low-malignant neoplasm.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Gentofte University Hospital, Hellerup, Denmark.

ABSTRACT
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destruction of the lungs. We present an unusual radiological presentation of lymphangioleiomyomatosis in a patient followed for 33 years with profuse coarse lung nodules in addition to the classical cystic lesions. We believe that this report might support the case for considering LAM a low-malignant neoplasm.

No MeSH data available.


Related in: MedlinePlus