Pulmonary Hemangiomas of Infancy and Childhood Report of Two Cases and Review of the Literature
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Cavernous hemangiomas are benign vascular tumors most commonly seen in the head and neck region in childhood. They have been described rarely in the lungs. Nosotros describe a patient with incidental pulmonary nodules discovered at autopsy, which measured up to 0.ix cm and which were nowadays in the lung parenchyma, likewise equally on the pleura. The nodules were composed of dilated vascular spaces lined by flattened bland cells. Immunohistochemical studies of the lining cells revealed CD34 and factor Viii immunoreactivity, consistent with a lesion of endothelial origin. Taken together, the gross, microscopic, and immunohistochemical findings support the diagnosis of multiple pulmonary cavernous hemangiomas.
Hemangiomas account for vii% of all benign tumors and are amongst the most common neoplasms in infants and children. The number of hemangiomas identified in adults is far fewer. Clangorous hemangiomas are a relatively uncommon variant, characterized by sharply divers, yet unencapsulated masses of blood-filled spaces supported past scant connective tissue that occasionally contains polish muscle. They are oft located in the skin, subcutaneous tissues, and liver.i Although these lesions can affect whatever visceral organ, they very rarely occur as a primary tumor of the lung.two Few cases of this entity have always been reported, and we identified only 1 immunohistochemically confirmed case of multiple pulmonary cavernous hemangiomas (PCHs) in the literature.3 While most cavernous hemangiomas are clinically benign, adverse clinical findings and emergent scenarios2,4–6 have been reported, making recognition of this lesion important in the differential diagnosis of pulmonary masses. We report a case of multiple PCHs in an 84-year-one-time man and describe the gross, histologic, and immunohistochemical features.
REPORT OF A Instance
An 84-year-old man with a history of chronic poliomyelitis with left hemiparesis and non–insulin-dependent diabetes mellitus presented in mid-July 2000 with a 48-60 minutes history of repeated falls. Subsequently all-encompassing workup, including a chest radiograph showing a correct centre pulmonary lobe infiltrate, the patient was treated for presumptive pneumonia. In the hours after access, the patient's neurologic condition improved; however, 24 hours after admission, the patient was plant unresponsive and he died.
At dissection, the patient was noted to have cardiomegaly, severe aortic and mitral valve calcific disease, too equally diffuse atherosclerotic cardiovascular and coronary artery disease. Exam of the lungs revealed multiple, minor, hemorrhagic nodules diffusely distributed throughout all lobes.
MATERIALS AND METHODS
Lung tissue was fixed in 10% buffered formalin and embedded in paraffin. Initial sections taken from the tissue blocks were stained with hematoxylin-eosin. For immunohistochemical studies, boosted sections were cutting, deparaffinized, rehydrated, and so quenched with hydrogen peroxide. Antigen retrieval was performed as follows: for CD34, slides were incubated with Dako Epitope Buffer (DakoCytomation Carpinteria, Calif), and for factor VIII and CAM 5.ii, slides were incubated with Dako Proteinase G Buffer (Dako) in a steam bath at 95°C for 45 minutes. After equilibration in phosphate-buffered saline for fifteen minutes, the slides were placed on an autostainer (Dako) and stained with antibodies to CD34 (monoclonal; Dako; 1:twenty dilution), factor Viii (monoclonal; Dako; 1:50 dilution), and CAM five.2 (monoclonal; Becton Dickinson, San Jose, Calif; 1:6 dilution). Immunoreactivity was detected using Dako EnVision methods co-ordinate to the manufacturer's recommended procedures.
PATHOLOGIC FINDINGS
At autopsy, the lungs weighed 2100 m and were diffusely congested and edematous with foci of patchy pneumonia. Numerous well-circumscribed, crimson-bluish, hemorrhagic nodules measuring upward to 0.nine cm were noted. The lesions were present in all lobes and involved lung parenchyma extending to subpleural tissue (Figure, A). In that location was no gross evidence of necrosis and/or fibrosis surrounding the nodules. Microscopically, the nodules were unencapsulated collections of dilated vascular spaces, variably filled with blood and separated by limited connective tissue stroma (Figure, B). Balmy stromal hyalinization and thrombosis were also noted. A single layer of flattened endothelial cells lining the vascular spaces was strongly positive for CD34 (Figure, C) and factor 8 (endothelial markers) and negative for CAM 5.2 (epithelial marker) by immunohistochemistry. The lesions were therefore presumed to be vascular in origin. Taken together, the gross appearance, histologic features, and immunohistochemical reactivity of these tumors led to a diagnosis of multiple PCHs.
A, Cut surface of lung showing multiple hemorrhagic nodules in parenchyma and pleura. B, Dilated, blood-filled vascular spaces with thin connective tissue stroma (hematoxylin-eosin, original magnification ×100). C, Monoclonal antibody to CD34 highlights a unmarried layer of flattened endothelial cells lining the vascular spaces (original magnification ×100)
A, Cut surface of lung showing multiple hemorrhagic nodules in parenchyma and pleura. B, Dilated, blood-filled vascular spaces with thin connective tissue stroma (hematoxylin-eosin, original magnification ×100). C, Monoclonal antibiotic to CD34 highlights a single layer of flattened endothelial cells lining the vascular spaces (original magnification ×100)
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Comment
Clangorous hemangiomas of the lung are exceedingly rare.2,5 A review of the literature reveals that PCH affects a wide historic period range, with the youngest reported patient being 10 weeks old and the oldest, 61 years.three,v Generally, PCHs are lonely lesions, although a single instance with multiple masses has been reported.3 Histologically, cavernous hemangiomas are distinguished past the formation of large, dilated vascular channels separated by connective tissue stroma. Masses are sharply defined merely not encapsulated, are variably filled with blood, and may display intravascular thrombosis and dystrophic calcification.1,5 The current case demonstrated multiple nodules present throughout all lobes and pleura. Microscopic examination revealed well-delineated nodules composed of widely dilated vascular spaces lined past CD34-positive, cistron Viii– positive, keratin-negative endothelial cells. Additionally, limited, mildly hyalinized stroma and occasional thrombosis were noted.
Detection of cavernous hemangiomas has varied significantly in reported cases. Some lesions presented clinically with findings such as respiratory distress, cyanosis, and hemoptysis.iv–6 In these cases, symptomatic presentations seem to depend on the location of the PCH. In 1 such case,4 a 54-year-onetime human presented with massive hemoptysis secondary to a 4 × 3-cm PCH involving the apicoposterior and anterior segments of the left upper lobe. Mucosal irregularities were observed in the left main bronchus, indicating encroachment of the lesion on the airways. However, nearly cases are identified on routine imaging studies. Cases of both solitary7 and multiple3 PCH have been monitored for upwards to 20 years earlier definitive exploration was undertaken. In ane case,7 a 29-twelvemonth-old woman had an abnormal shadow on breast radiographs for 20 years before undergoing computed tomography–guided, video-assisted thoracoscopic surgery, which revealed a ane.0 × 1.0-cm PCH. In the case of multiple PCHs,3 a 61-year-old adult female with multiple "tumor" shadows on chest radiographs carried a diagnosis of metastatic tumor to lung with unknown primary for 10 years before dying of congestive centre failure. At dissection, multiple lesions of ane cm or less were noted and histologically confirmed as PCH. The patient described in this article showed no apparent agin symptomatology or consequence as a event of his lesions.
Surgical protocols indicate that solitary PCH should be treated with surgical excision,2 as illustrated in virtually cases in the literature.4,7,eight 1 group6 has reported using interferon alfa-2a to successfully treat a 7-year-old patient with a PCH associated with respiratory distress and hemoptysis. These cases suggest that recognition of PCH as part of the differential diagnosis of pulmonary lesions is important, as both surgical resection4,5,vii,8 and other treatments may be curative. Conversely, no intervention was undertaken in the previously reported instance of multiple PCH3 with no change in the size or number of the lesions over time. Additionally, 1 of the surgically excised cases had been monitored for 20 years with no symptoms or change in the lesion. These experiences propose that radiographic follow-upward may play a significant role in asymptomatic, stable, small lesions.
Since master vascular lung neoplasms are exceedingly rare, it is necessary to consider a range of entities in the differential diagnosis of PCH, taking into account the patient'southward age, clinical history, and symptomatology. In infants and young children, intracystic hemorrhage within a built cystic adenomatoid malformation may suggest a vascular lesion. Pulmonary arteriovenous malformations seen in Osler-Weber-Rendu syndrome are also detected in this population, while intrapulmonary hematomas associated with nativity trauma may merely exist suspected in the first few days of life.v In older children and adults, entities such as Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma must be excluded. While all 3 weather tend to present every bit multiple pulmonary nodules, specific clinical and histologic features may assistance in distinguishing these tumors from PCH. Kaposi sarcoma usually occurs in the setting of immunocompromised patients and is characterized by a spindle cell proliferation with mild to moderate nuclear atypia and a depression mitotic rate surrounding vascular slits filled with extravasated cerise claret cells. In the lung, Kaposi sarcoma may abound around large blood vessels or brandish a lymphatic distribution with widening of the alveolar septa,9 features not associated with PCH. Primary angiosarcomas of the lung are rare, and a metastatic lesion should be suspected.10 Histologically, angiosarcoma is characterized by atypical cells lining anastomosing vascular channels. While the sheetlike pattern of growth observed in high-grade lesions makes differentiation from PCH easier, rare cases with widely dilated anastomosing spaces mimicking cavernous hemangioma have been reported,11 making the presence or absence of cellular atypia the key feature in diagnosis. Epithelioid hemangioendothelioma is also a rare, often multifocal vascular neoplasm characterized by proliferation of round to oval cells with arable eosinophilic cytoplasm and big nuclei, embedded in a myxoid or hyaline stroma. Although epithelioid hemangioendothelioma may exhibit tumor cells with big intracytoplasmic vacuoles, these spaces should non be confused with the expanded vascular channels identified in PCH.12
In summary, although the lung is invested with a dual blood supply and rich vascular network, vascular lesions such equally PCH are extremely rare. Pulmonary cavernous hemangioma tin be asymptomatic or can present with life-threatening symptoms, warranting its consideration in the differential diagnosis of pulmonary lesions. The instance of multiple PCHs reported here represents a clinically undetected lesion discovered at autopsy. Microscopic examination and immunohistochemical studies revealed well-delineated nodules equanimous of widely dilated spaces lined by endothelial cells, consequent with a diagnosis of clangorous hemangioma. Surgery and/or close radiographic follow-up remain the handling of choice in clinically or radiographically detected lesions.
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The authors take no relevant financial interest in the products or companies described in this article.
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