Pulmonary Sclerosing Hemangioma Research Articles

Diagnosis and treatment for pulmonary sclerosing hemangioma

背景与目的肺硬化性血管瘤(pulmonary sclerosing hemangioma, PSH)概念的提出迄今只有50余年，是一种少见的肺部良性疾病。PSH临床表现有一定特点，需与肺癌鉴别诊断。本文总结我院48例PSH的临床诊治经验并复习文献，旨在提高对PSH的认识，探讨合理的诊断与治疗手段。方法2001年1月-2011年4月共收治PSH 48例，结合文献报道总结分析PSH的发病特点、临床表现、影像学、病理学特点及预后。结果全组48例，无症状者27例(56.3%)，肿物大小0.2 cm-7.0 cm，平均2.1 cm，各个肺叶均有发生。合并肺门或纵隔淋巴结肿大者15例(31.3%)。手术47例，其中肺叶部分切除29例(61.7%)，肺叶切除14例(29.8%)，肿物剔除3例(6.4%)，前纵隔肿物切除者1例(2.1%)。47例术后均无复发。CT引导下穿刺活检诊断1例，随访28个月未见肿瘤进展。结论PSH术前定性诊断困难，手术既是确诊手段又是有效的治疗手段，该病预后良好。

FDG–PET imaging findings of a pulmonary sclerosing hemangioma

Pulmonary sclerosing hemangiomas are generally regarded as benign lung lesions arising from type II pneumocytes and bronchial epithelium. In some cases malignant features may be present. There are several case reports describing the radiographic, computed tomography (CT), and magnetic resonance imaging (MRI) characteristics of pulmonary sclerosing hemangiomas. Given the potential for low-grade malignancy 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) imaging may be useful in the diagnostic work up and therapeutic planning in these patients, however the FDG–PET imaging features of pulmonary sclerosing hemangiomas are not well defined. We report a case of histopathologically diagnosed sclerosing hemangioma of the lung which demonstrated intermediate uptake of FDG on the preoperative PET/CT evaluation.

Comparison of genetic and epigenetic alterations at 11 tumor suppressor loci in pulmonary sclerosing hemangioma and adenocarcinoma

ABSTRACTPulmonary sclerosing hemangioma (SH) is an unusual tumor of pneumocytic origin. Morphologically, SH can mimic pulmonary adenocarcinomas. Here, the authors compared genetic and epigenetic aberrations in SH with those in pulmonary adenocarcinoma. Clinicopathologic characteristics, microsatellite alterations, and CpG island methylation were analyzed in pulmonary SHs (n = 24) and adenocarcinomas (n = 34) to compare their patterns of molecular abnormalities. SHs were also analyzed immunohistochemically to characterize the expression status of proteins involved in basic biologic processes. The clinical presentation of SH cases was generally benign. Both cell types of SH stained positive for thyroid transcription factor 1 (TTF-1), epithelial membrane antigen (EMA), β-catenin, E-cadherin, and vascular endothelial growth factor (VEGF). Allelic imbalances in D3S1283, D3S1234, D3S1300, D3S1285, TP53, D17S938, and D9S179 were less frequent in SH than in adenocarcinoma; rates of allelic imbalances in D20S170 and D21S1446 were not significantly different. In SH, CpG island methylation frequencies of p16INK4a (0.0%) and RASSF1A (12.5%) were significantly lower than those in adenocarcinoma (29.4% and 38.2%, respectively); the frequencies of HOX D9, D11, and D13 gene methylation in SH were 37.5%, 33.3%, and 33.3%, respectively. The results show that pulmonary SH and adenocarcinoma share similar genetic and epigenetic aberrations, but also exhibit significant differences, especially in tumor suppressor genes.

Pulmonary sclerosing hemangioma in a 21-year-old male with metastatic hereditary non-polyposis colorectal cancer: Report of a case

BackgroundPulmonary sclerosing hemangioma (SH) is a rare tumor of the lung predominantly affecting Asian women in their fifth decade of life. SH is thought to evolve from primitive respiratory epithelium and mostly shows benign biological behavior; however, cases of lymph node metastases, local recurrence and multiple lesions have been described.Case PresentationWe report the case of a 21-year-old Caucasian male with a history of locally advanced and metastatic rectal carcinoma (UICC IV; pT4, pN1, M1(hep)) that was eventually identified as having hereditary non-polyposis colorectal cancer (HNPCC, Lynch syndrome). After neoadjuvant chemotherapy followed by low anterior resection, adjuvant chemotherapy and metachronous partial hepatectomy, he was admitted for treatment of newly diagnosed bilateral pulmonary metastases. Thoracic computed tomography showed a homogenous, sharply marked nodule in the left lower lobe. We decided in favor of atypical resection followed by systematic lymphadenectomy. Histopathological analysis revealed the diagnosis of SH.ConclusionsCases have been published with familial adenomatous polyposis (FAP) and simultaneous SH. FAP, Gardner syndrome and Li-Fraumeni syndrome, however, had been ruled out in the present case. To the best of our knowledge, this is the first report describing SH associated with Lynch syndrome.

Coexistence of pulmonary sclerosing hemangioma and primary adenocarcinoma in the same nodule of lung

Pulmonary sclerosing hemangiomas (PSH) of the lung are uncommon tumors and may present cytological atypia with unusual manifestations. The development of PSH combined with other different tumors in lung is extremely rare. We report a case of coexistence of PSH and primary adenocarcinoma in a young female occurring in the same pulmonary nodular mass of right lower lobe. The solitary mass of lung was well-circumscribed on chest computed tomography (CT) and gross examination. Histologically, the mass contained two separated portions and displayed typically histological features of PSH and acinar adenocarcinoma, respectively. In PSH portion, the tumor was composed of sheets of round cells with scattered surface cuboidal cells forming small tubules. Both round and surface cells were diffusely positive for epithelial membrane antigen (EMA) and thyroid transcription factor-1 (TTF-1), but lack immunoreactivity for pancytokeratin in round cells. In adenocarcinoma portion, the tumor cells formed irregular-shaped glands with cytologically malignant cells infiltrating in fibroblastic stroma, and no TTF-1-positive round cells could be observed in this portion. Under the microscopy, there was no gradual transition of these two portions observed in mass. A diagnosis of PSH combined with primary adenocarcinoma of lung was made. There was no evidence of tumor recurrence during the period of postoperative 6-month follow-up. To our knowledge, this is the first case of coexistence of PSH and adenocarcinoma in the same nodule of lung. In addition, the biological behavior and histological differential diagnosis of this tumor were also discussed.

Sixteen cases of pulmonary sclerosing haemangioma: CT findings are not definitive for preoperative diagnosis

To describe and assess computed tomography (CT) imaging findings of pathologically confirmed pulmonary sclerosing haemangioma (PSH), a rare benign tumour, in 16 patients. This study was approved by the human investigation committees of the two participating institutions, and the requirement of informed consent was waived. Findings from CT examinations in 16 patients with pathologically confirmed PSH were retrospectively reviewed. Two radiologists in consensus assessed the location, size, contour, and border of nodules, as well as the enhancement patterns. The study comprised 16 patients (two male and 14 female) aged 30-70 years. PSHs appeared as well-defined, round or ovoid masses with a diameter of 2.2±0.3cm, and were generally demonstrated as juxtapleural masses (94%) on CT. Calcification (13%), the air crescent sign (13%), and a prominent pulmonary artery (25%) in the tumours were also demonstrated in the cohort. The mean tumour attenuation value at CT was 30±3HU before intravenous administration of contrast media, and was significantly lower than that of the enhanced phase (79±3HU, p<0.05). Twelve tumours (75%) enhanced homogeneously compared with four tumours (25%) which enhanced heterogeneously. The diameters of the heterogeneously enhanced tumours were larger than those of the homogeneously enhanced tumours (p<0.05). The relatively characteristic CT findings include a markedly contrast-enhanced juxtapleural mass with homogeneous enhancement for the smaller tumours (<3cm in diameter) or heterogeneous enhancement for the larger tumours (>3cm). However, CT findings are not definitive for preoperative diagnosis of PSH.

F-18 FDG PET/CT in Evaluation of Pulmonary Sclerosing Hemangioma

Pulmonary sclerosing hemangioma (PSH), or the alternative name of "sclerosing pneumocytoma," is a rare benign neoplasm. PSH is often asymptomatic and presents as a solitary pulmonary nodule on radiologic imaging studies. Few articles have been reported to describe the FDG PET findings about PSH. We reported FDG PET findings of 6 cases of PSH and reviewed the literatures. FDG PET and PET/CT imaging studies of 6 patients with proven PSH were reviewed. The relation between the degree of FDG uptake and tumor size was analyzed. All 6 tumors were detected by FDG PET or PET/CT. Mean diameter of these tumors was 2.9 cm. Mean standard uptake value was 2.45 (range, 1.8-3.93). The maximal standard uptake value of FDG uptake was positively correlated with the tumor size (R = 0.9694, P < 0.05). PSH demonstrates low to moderate uptake on FDG PET imaging. The larger PSH has higher uptake and might be misrecognized as a malignancy. The possibility of false-positive result caused by PSH should be considered while interpreting FDG PET images of solitary pulmonary nodules.

A Case of Pulmonary Sclerosing Hemangioma Surrounded by Giant Cystic Change on High-resolution Computed Tomography

Pulmonary sclerosing hemangioma is a rare benign neoplasm, usually presenting as a well-defined round or oval mass. We report a case of pulmonary sclerosing hemangioma surrounded by an unusual giant cystic change on high-resolution computed tomography, which led to the suspicion of this tumor.

Pulmonary sclerosing hemangioma: pitfalls in management

Pulmonary sclerosing hemangioma is a rare benign tumor for which surgical excision is curative with an excellent prognosis. It often mimics malignancy, making it a diagnostic and management challenge. We reviewed the clinical, radiological, and histopathological characteristics as well as the surgical management of our patients to assess the potential pitfalls in management. All 19 patients who underwent surgical biopsy between January 1999 and December 2009 with a final histopathological diagnosis of pulmonary sclerosing hemangioma were reviewed retrospectively. The median age was 48 years, 89.5% were female, 89.5% were Chinese, and 68.4% were asymptomatic. There were no specific diagnostic radiological characteristics; 1 of 3 patients who underwent combined positron-emission and computed tomography had a false-positive result. Preoperative attempts at establishing a diagnosis were successful in 20% of patients who had invasive procedures. Intraoperative frozen-section evaluation for pulmonary sclerosing hemangioma had an error rate of 25% and a deferred rate of 31%, resulting in nearly half of our patients undergoing unnecessary extensive surgical procedures. We hope to heighten awareness of this diagnosis, thereby increasing the index of suspicion and minimizing errors in diagnosis.

Clinical evaluation of pulmonary sclerosing hemangioma

Clinical evaluation of pulmonary sclerosing hemangioma

Pulmonary sclerosing haemangioma presented like intra-lobar sequestration

Fig. 1. (A) This contrast computed tomography chest scan shows an oval heterogeneously enhancing mass with abundant peripheral vascularity of size 5.3 cm 3.5 cm 4.6 cm noted in the lateral basal segment of right lower lobe. Punctate calcification noted within the mass. Alveolar opacities noted in right lower lobe lateral, medial and posterior basal segments. (B) This spiral angiographic computed tomography chest scan shows the vascular ovoid mass lesion in the right lung lower lobe fed by an anomalous artery from right renal artery. Venous drainage noted into right inferior pulmonary vein. The above features are suggestive of right lower lobe intra-lobar sequestration.

Surgery for Pulmonary Sclerosing Hemangioma: Lobectomy versus Limited Resection

BackgroundPulmonary sclerosing hemangioma is a rare thoracic tumor, and pathophysiology or clinical course of this tumor is not yet fully described. Furthermore, there is no consensus on the standard operative procedure for this tumor.Material and MethodsMedical records of thirty-two patients, who underwent surgical resection of pulmonary sclerosing hemangioma from 1996 to 2007, were retrospectively reviewed.ResultsNineteen patients underwent lobectomy and thirteen patients underwent limited resection. Video-assisted thoracoscopic surgery was performed in 9 patients in the latter group. Lymph node dissection was done in 21 patients, and one patient was found to have lymph node metastasis of the tumor. There was no postoperative complication, no early death and no tumor-related late mortality. The mean follow-up duration was 39.3 months (2 months~129 months), and all patients were free of local recurrence and distant metastasis during this period. There was no significant difference in patient's characteristics between the two groups, except that the mean hospital stay was shorter in limited resection group than in lobectomy group (p=0.0031).ConclusionPulmonary sclerosing hemangioma usually requires surgical resection for both diagnosis and treatment. Limited resection can decrease hospital stay with a surgical outcome comparable to lobectomy, and may be preferred to lobectomy if sufficient resection margin can be achieved.

Pulmonary sclerosing hemangioma with pleural dissemination: Report of a case

Pulmonary sclerosing hemangioma is relatively rare and is usually considered a benign tumor. There have been no reports of pulmonary sclerosing hemangioma with pleural dissemination. This report presents an extremely rare case of pulmonary sclerosing hemangioma with pulmonary dissemination. A 57-year-old woman was found to have an abnormal shadow in the right lower lung field on chest X-ray. Chest computed tomography (CT) indicated a 2.5-cm mass in the right lower lobe. A bronchoscopic biopsy failed to identify malignant cells, which led to the patient undergoing an excisional lung biopsy. Intraoperative findings showed a tumor in the right lower lobe with multiple small nodules in the pleura. The pathological findings revealed that the tumor was sclerosing hemangioma with pleural dissemination. Annual follow-up CT showed irregular pleural thickness, which suggested progressive dissemination 3 years after the operation. Although pulmonary sclerosing hemangioma is regarded as a benign tumor, the potential for malignancy may be a consideration in this setting.

CT image morphology features of pulmonary sclerosing hemangiomas

The aim of the study was to analyze the CT morphology features of pulmonary sclerosing hemangiomas (PSHs) and improve the diagnosis ability of this disease. The 18 cases of pulmonary sclerosing hemangioma (PSH) confirmed by operation and histopathology from August 2002 to May 2009 were collected, including 17 females and 2 males, aged from 19 to 60 years old, with an average age of 43 years. All the cases underwent plain CT scan, among them, 16 cases received enhanced CT scan. The 18 cases had isolated mass. Mean long-axis diameter of these lesions was (2.7 ± 1.3) cm (range, 1.9–4.2 cm). Of all cases, 5 cases (27.8%) were round in shape, 9 cases (50%) were oval, 4 cases (22.2%) were lobulated, and 14 cases (77.8%) were smooth margin. The air meniscus sign was in 2 cases (11.1%), and the halo sign in 3 cases (16.7%). Two cases (11.1%) contained small nodular calcification, the remaining 16 cases (70%) were homogeneous density, the CT density of the masses ranged from 24–47 HU, and the mean value was 35 HU. Sixteen cases received enhanced scan, the welt vessel sign was in 8 cases (44.4%),1 case showed less enhancement, 5 cases showed marked homogeneous enhancement and 10 cases showed intense and patchy heterogeneous enhanced. The CT density of the enhancing masses ranged from 60–110 HU, the mean value was 35 HU, and the net enhancement value was 14–80 HU, the mean value was 55 HU. PSH should be considered in middle-aged female whose CT found that single round or oval pulmonary nodules, with smooth margin, or associated with the air meniscus sign, the halo sign, or the marked enhancement.

Large Masses With Diffuse Calcification: Pulmonary Sclerosing Hemangioma

A 46-year-old man was incidentally found at evaluation for eye surgery to have two pulmonary masses on a chest roentgengram. The computed tomographic scan revealed two irregular, lobulated masses with calcification in the right upper lobe. One mass was located near the hilum of the lung (Fig 1A), and the other more peripherally (Fig 1B). There was no uptake on fluorodeoxyglucose positron emission tomographic scan.

Pulmonary sclerosing hemangioma with atypical radiologic findings

Abstract Sclerosing hemangioma of the lung is an uncommon benign tumour that usually presents as an asymptomatic solitary nodule and affects middle age women. Because there are few findings on radiologic studies that are characteristic of sclerosing hemangioma, it is difficult to diagnose on the basis of imaging and biopsy remains the definitive diagnostic test. We report a case of pulmonary sclerosing hemangioma with extremely unexpected imaging findings and review the literature.

Determination of clonal status of pulmonary sclerosing hemangioma with X-chromosome inactivation mosaicism and polymorphism of phosphoglycerate kinase and androgen receptor genes

It has been reported that both polygonal and cuboidal cells in pulmonary sclerosing hemangioma (PSH) are monoclonal in origin and represent a variable differentiation from a common progenitor cell. However, it remains unclear about the clonality of the entire PSH lesion composed of these two types of cells. Thus, we analyzed the clonality of 22 cases of PSH and the relationship between the entire PSH and two types of cells using laser microdissection and X-chromosomal inactivation mosaicism and polymorphism at the phosphoglycerate kinase (PGK) and androgen receptor (AR) loci in female somatic cells. The results demonstrated all 22 cases of PSH showed typical histopathologic characteristics, including characteristic round or polygonal cells within the stroma and surface cuboidal cells lining the papillary projections or cystic spaces. The rates of polymorphism were 31.8% (7/22) and 86.3% (19/22) for the PGK and AR gene, respectively. After digestion by Hpa II or Hha I, one of two PCR amplification bands disappeared from all the samples, while the other band was retained, indicating neoplastic characteristics. Thus, we concluded that the entire PSH lesion, polygonal and cuboidal cells were neoplastic hyperplasia and originated from a common progenitor cell.

Positive expression of c-myc and p53 products in two cases of pulmonary sclerosing hemangioma

The c-myc and p53 genes are thought to be an oncogene and a tumor suppressor gene, respectively. These genes' products are characteristic of malignant tumors. We quantitatively analyzed the c-myc and p53 products by flow cytometry in two cases of pulmonary sclerosing hemangioma. In case 1, 32.3% of the tumor cells were found to have the c-myc product, and 8.9% were found to have the p53 product. In case 2, 6.7% of the tumor cells were found to have the c-myc product and 15.5% were found to have the p53 product. The percentages in both cases were twice as high as those in a negative control lymphocytes stained with c-myc and p53 products. Therefore, these two cases showed positive expression of the c-myc and p53 products. In addition DNA from six other patients with sclerosing hemangioma was analyzed with paraffin-embedded sections. All six had DNA diploidy, with DNA indexes ranging from 0.91 to 1.03 and coefficients of variation ranging from 3.0 to 5.5. We suggest that pulmonary sclerosing hemangioma is a very weakly malignant tumor.

A Pulmonary Sclerosing Hemagioma with an Increasing Uptake on PET

Pulmonary sclerosing hemangioma is a relatively rare neoplasm of the lung. Although there have been five previous case reports that address the 18-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) findings in a sclerosing hemangioma of the lung, no report has demonstrated an interval change in the FDG-PET findings. This report describes a case of pulmonary sclerosing hemangioma which presented with an increase of uptake of FDG-PET after about one years' follow-up.

Cytogenetic study of a pulmonary sclerosing hemangioma

Pulmonary sclerosing hemangioma (PSH) is an uncommon benign tumor that presents as a solitary asymptomatic and slow-growing nodule. It occurs in both young and old persons; peak incidence is in the fifth decade. Both sexes are affected by this tumor, but women more frequently than men. On histological examination, PSH shows prominent sclerotization and vascularization of the tissue. Recent studies conclude that PSH derives from type II pneumocytes, but the potential for progression and histogenesis remains controversial. We report a case of pulmonary sclerosing hemangioma in a 61-year-old woman with a neoplastic node 1 cm in diameter. The karyotype was 46,XX,t(8;18),der(14;15),+14 in all the cells analyzed. PTEN (10q23) and IgH (14q32) probes were analyzed in interphase nuclei and paraffin-embedded tissues of tumor cells. These chromosome abnormalities could provide information about the relationship of genetic changes to the biological properties of sclerosing hemangioma tumors.