The two major types of cells of pulmonary sclerosing haemangioma (PSH) with the same origin show significant differences in morphological phenotype. Whether these differences are caused by their different differentiation status is still uncertain. The aim of this study was to analyse their differentiation status by detecting the expression of several stem cell markers in PSH.
The expression of stem cell markers was examined by using streptavidin peroxidase (SP) immunohistochemisty in 45 PSH specimens. Also, the two types of cells were, respectively, captured by laser capture microdissection (LCM) from 28 PSH specimens, and total RNA was then extracted followed by reverse transcription-polymerase chain reaction (RT-PCR). The results demonstrated that the expression rates of ABCG2, Notch1 and Notch3 in polygonal cells were significantly higher than those in cuboidal cells (P < 0.05), and the expression levels of ABCG2, Notch3 and Jagged1 in polygonal cells were clearly higher than those in cuboidal cells (P < 0.05).
The data obtained provided evidence that the two types of cells in PSH may be different in differentiation status. The differentiation difference between the two types of cells might lead to variation in their morphological phenotype.
"The hypothesis is made based upon following observations in our case: 1) although morphologically distinctive, the two different components are so well mingled that they cannot be distinguished either by radiology or gross examination; 2) intimate mixture of PSH and extensive PNC lesions are present throughout the entire multi-nodular lesion; 3) the two different components both express TTF-1 (a tissue-specific transcription factor expressed by type II pneumocytes and Clara cells). This ‘one tumor’ theory was further suggested by previous studies demonstrating different phenotype and differentiation status of the two types of PSH cells and neuroendocrine immunoactivity and neurosecretory granules in nested cells of PSH [6-8,17]. In addition, the recent finding that PNCs share a common lineage with alveolar cells  may further support that possibility that this rare ‘third’ cell type and the other two may originate from the same progenitor. "
[Show abstract][Hide abstract] ABSTRACT: We encountered an extremely rare case of multiple pulmonary sclerosing hemangiomas (PSH) with extensive neuroendocrine lesions, including pulmonary neuroendocrine cell (PNC) hyperplasia, multiple carcinoid tumorlets and typical carcinoid tumors within one pulmonary lobe. To the best of our knowledge, this is the first reported case in the English medical literature of PSH combined and admixed with carcinoid tumors and extensive neuroendocrine proliferation. This case is noteworthy for several reasons. First, the lesion is multi-nodular and unusually large for a typical PSH, which may mimic malignancy on imaging studies and cause diagnostic difficulties. Second, sampling bias may lead to diagnostic errors for a lesion containing two different types of neoplasms. Third, our case displays a rare mixed and mosaic pattern of PSH with a full spectrum of pulmonary neuroendocrine lesions, which may imply a potential intrinsic association in pathogenesis between PSH and concomitant neuroendocrine neoplasms. The clinical implication of multiple PSHs is also discussed.
World Journal of Surgical Oncology 07/2014; 12(1):209. DOI:10.1186/1477-7819-12-209 · 1.41 Impact Factor
"Although the nature of PSH is still controversial, its histological feature has been well described. Histologically, PSH contains surface cuboidal cells and polygonal cells, and these two types of cells typically form four architectural patterns including papillary, sclerotic, solid, and hemorrhagic
[6,7]. The surface cuboidal cells resemble normal type II pneumocytes while the polygonal cells are diffusely distributed in stroma, and have no counterparts in normal lung tissue. "
[Show abstract][Hide abstract] ABSTRACT: Pulmonary sclerosing hemangioma (PSH) is an uncommon pulmonary tumor. Histologically, PSH typically consists of two types of cells, surface cuboidal cells and polygonal cells, four architectural patterns including papillary, sclerotic, solid, and hemorrhagic. Herein, we present a case of PSH in a 59-year-old Chinese female. The tumor was predominantly composed of solid area presenting with diffuse spindle cells rather than polygonal cells. Focally, classical papillary and sclerotic area could be seen. Immunohistochemical staining showed that the spindle cells were positive for TTF-1, EMA, Actin(SM) and Vimentin, and negative for cytokeratin, cytokeratin7, cytokeratin5/6, surfactant apoprotein A, surfactant apoprotein B, CD34, CD99, S-100, HMB45, Desmin, Synaptophysin, CD56, ALK and Calretinin. The immunophenotype of the dense spindle cells in this case was similar to that of the polygonal cells, and thus the spindle cells may be the variants of polygonal cells. Based on morphologic features and the immunohistochemical profile, the tumor was diagnosed as a PSH. The significance of spindle cells change is unclear for us. To our knowledge, this is the first reported case of PSH showing dense spindle cells in solid area. This case represents a potential diagnostic pitfall, as it may be misdiagnosed as a mesenchymal tumor such as inflammatory myofibroblastic tumor, synovial sarcoma, solitary fibrous tumor, leiomyoma, or even mesothelioma, especially if the specimen is limited or from fine- needle aspiration.Virtual slidesThe virtual slide(s) for this article can be found here:
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