TY - JOUR
T1 - Clear-cell proliferation of the lung with lymphangioleiomyomatosis-like change
AU - Pileri, S. A.
AU - Cavazza, A.
AU - Schiavina, M.
AU - Zompatori, M.
AU - Pederzoli, M.
AU - Goldfischer, M.
AU - Sabattini, E.
AU - Ascani, S.
AU - Pasquinelli, G.
AU - Bonetti, F.
AU - Colby, T. V.
PY - 2004/2
Y1 - 2004/2
N2 - Aims: To describe two cases of a peculiar pulmonary lesion, which expand both the morphological and the immunophenotypic spectrum of perivascular epithelioid cell (PEC)-related disorders. Methods and results: One man and one female, with and without the tuberous sclerosis complex (TSC), respectively, showed pulmonary cysts and small nodules on computed tomography scan. In the former, lymphangioleiomyomatosis (LAM) was suspected. In both cases, an open lung biopsy was performed, whose cut surface displayed numerous cysts lined by thin/thick septa. Microscopically, the septa were associated with micronodular or interstitial proliferation of medium/large-sized elements with abundant clear (periodic acid-Schiff-positive/diastase-sensitive) cytoplasm and distinct cell borders, embedded in fibrous tissue. The elements were CD34+, vimentin-positive and, to a lesser extent, HMB-45+ and MART-1+. The stains for specific muscle actin, desmin, S100 protein, CD31, FVIIIRAg, cytokeratins, CD45, CD68, oestrogen and progesterone receptors were all negative. Ki67 labelling was <1%. Electron microscopy displayed cytoplasmic vacuoles containing glycogen particles. The TSC1 and TSC2 gene status could not be assessed because of poor DNA preservation. In the man with TSC, a focus of micronodular pneumocyte hyperplasia was also found. Conclusions: Because of the coexpression of CD34 and melanoma-associated antigens and the occurrence of TSC in one patient, the cases described here add a new piece to the puzzle of PEC lesions and contribute to the open discussion on the origin of LAM and LAM-like proliferations.
AB - Aims: To describe two cases of a peculiar pulmonary lesion, which expand both the morphological and the immunophenotypic spectrum of perivascular epithelioid cell (PEC)-related disorders. Methods and results: One man and one female, with and without the tuberous sclerosis complex (TSC), respectively, showed pulmonary cysts and small nodules on computed tomography scan. In the former, lymphangioleiomyomatosis (LAM) was suspected. In both cases, an open lung biopsy was performed, whose cut surface displayed numerous cysts lined by thin/thick septa. Microscopically, the septa were associated with micronodular or interstitial proliferation of medium/large-sized elements with abundant clear (periodic acid-Schiff-positive/diastase-sensitive) cytoplasm and distinct cell borders, embedded in fibrous tissue. The elements were CD34+, vimentin-positive and, to a lesser extent, HMB-45+ and MART-1+. The stains for specific muscle actin, desmin, S100 protein, CD31, FVIIIRAg, cytokeratins, CD45, CD68, oestrogen and progesterone receptors were all negative. Ki67 labelling was <1%. Electron microscopy displayed cytoplasmic vacuoles containing glycogen particles. The TSC1 and TSC2 gene status could not be assessed because of poor DNA preservation. In the man with TSC, a focus of micronodular pneumocyte hyperplasia was also found. Conclusions: Because of the coexpression of CD34 and melanoma-associated antigens and the occurrence of TSC in one patient, the cases described here add a new piece to the puzzle of PEC lesions and contribute to the open discussion on the origin of LAM and LAM-like proliferations.
KW - Clear-cell 'sugar' tumour of the lung
KW - Lymphangioleiomyomatosis
KW - Molecular biology
KW - Morphology
KW - Perivascular epithelioid cell
KW - Phenotype
KW - Tuberous sclerosis complex
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U2 - 10.1111/j.1365-2559.2004.01788.x
DO - 10.1111/j.1365-2559.2004.01788.x
M3 - Article
C2 - 14764059
AN - SCOPUS:10744233732
SN - 0309-0167
VL - 44
SP - 156
EP - 163
JO - Histopathology
JF - Histopathology
IS - 2
ER -