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【肺腫瘍】孤立性肺毛細血管腫(Solitary Pulmonary Capillary Hemangioma):GGOを示す良性腫瘍

Solitary Pulmonary Capillary Hemangioma of Adult Cases: Clinicopathologic Characteristics as an Unrecognized Entity

Am J Surg Pathol. 2016 Oct;40(10):1380-9.

以前から肺高血圧と関連する肺毛細血管腫症 pulmonary capillary hemangiomatosis(PCH)という概念が知られていたが、PCH に類似した単発性腫瘤を呈する疾患が 2000 年に報告された(最初の論文は記事の後半に掲載)。その後多数の論文が出て、日常診療でも稀に経験される疾患として知られつつある。

Abstract
Solitary pulmonary capillary hemangioma (SPCH) is a rare disease, first described about autopsy cases in 2000 and about surgically resected cases in 2006. To date, only 9 surgically resected cases have been published in English. Here, we report 7 original cases with surgery (median age, 54 y; 4 females, 2 never-smokers). All patients were asymptomatic, and all nodules were detected by computed tomography (CT). The median (range) size of nodule was 11 (8 to 16) mm. Six of 7 cases showed the part-solid nodule appearance and 1 showed pure ground-glass nodule appearance in CT findings. The growth speed was very slow. No abnormal uptake of fluorine-18 fluorodeoxyglucose was observed in systemic positron-emission CT in all 3 cases we examined. No patients died from SPCH. Histologically, SPCH manifested as a solitary lesion composed of densely proliferating and dilated capillaries without cytologic atypia within the alveolar septa. In addition, capillaries of SPCH spread into the vascular lumen and involved the walls of bronchioles with protrusion into the lumen. Immunohistochemically, capillaries of SPCH uniformly expressed endothelial markers, such as CD31, CD34, and Factor VIII; and α-smooth muscle actin positive cells were also observed. To be accurately diagnosed, especially in intraoperative frozen sections, SPCH should be conceived as an entity that presents as a solitary nodule in CT. We propose that SPCH is an unrecognized benign capillary proliferative disease.

僕自身は今まで2例診断したことがあり、過去症例も合わせると自分でみたのは4例である。稀だが、すごく稀というほどでもないのだろう。画像上は GGO/GGN を呈するため原発性肺腺癌にみえるのだが、肉眼所見および組織所見は全く異なっている。特徴的なので、一度みたことがあれば、忘れないだろう。

【SANOTIC SUMMARY】
1.大きさは 8~16 mm 程度で、part-solid nodule を呈することが多いが、pure GGN を呈する例もある。
2.死亡例はない。
3.肺胞隔壁内で拡張した毛細血管が密に増殖し、一部周囲にも進展する。

PubMed のリンクはこちら👇


【最初の報告】Pulmonary capillary hemangiomatosis-like foci. An autopsy study of 8 cases

Am J Clin Pathol. 2000 May;113(5):655-62.

Abstract
Pulmonary capillary hemangiomatosis (PCH) typically occurs in young patients who have signs and symptoms of pulmonary hypertension. It commonly is misdiagnosed in life as pulmonary veno-occlusive disease, and the correct diagnosis usually is not made until autopsy. Autopsy records, including reports, gross photographs, histologic slides, clinical histories, and radiographic images, were reviewed to identify cases with morphologic changes characteristic of PCH. The previous case reports describe PCH as a diffuse process throughout both lung fields. All patients were symptomatic, and most died of the disease. This article details 8 cases of PCH-like foci that were incidental findings at autopsy in which the patients did not have symptoms of pulmonary hypertension nor did PCH contribute in any way to death. This is the first case series that describes pathologic changes of PCH occurring in this setting, and we hope to provide more interest in PCH and its natural history.

PubMed のリンクはこちらから👇

PDF のダウンロードはこちらから👇


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