Pulmonary sclerosing hemangioma associated with familial adenomatous polyposis

Kansai Medical University, Moriguchi, Ōsaka, Japan
Human Pathlogy (Impact Factor: 2.81). 07/2004; 35(6):764-8. DOI: 10.1016/j.humpath.2004.02.003
Source: PubMed

ABSTRACT A 45-year-old woman presented with asymptomatic solid tumor in the lower right lobe of the lung. Histologically, the tumor comprised a monolayer of surface cells and round stromal cells displaying sclerotic areas. Immunohistochemical studies suggested a diagnosis of sclerosing hemangioma. Interestingly, morular lesions were also observed. Analyses of the gastrointestinal (GI) tract showed mild familial adenomatous polyposis (FAP) and numerous fundal gland polyps, indicating attenuated FAP (AFAP). All components of the sclerosing hemangioma displayed aberrant nuclear and cytoplasmic expression of beta-catenin. However, such findings were much weaker in adenomas of the GI tract and were barely observed in fundal gland polyps. These results strongly suggest an association between sclerosing hemangioma and the AFAP. To the best of our knowledge, this is only the second report of lung tumor associated with FAP and is the first describing an association with sclerosing hemangioma. A new category of FAP-associated lung tumors may be indicated.

  • [Show abstract] [Hide abstract]
    ABSTRACT: Familial adenomatous polyposis (FAP) is characterised by colonic and duodenal adenomatous polyps that carry a risk of malignant transformation. Malignant degeneration of duodenal adenomas is difficult to detect. We speculated that 2-((18)F)-fluoro-2-deoxy-D: -glucose positron emission tomography (FDG-PET) might be able to detect early duodenal cancer in FAP. Accordingly, we investigated the role of FDG-PET in the management of FAP patients. FDG-PET was performed in 24 FAP patients. Eight had advanced duodenal adenomas (Spigelman IV), including two patients with duodenal cancer. Scans were defined as positive on the basis of focal FDG accumulation. Pathological FDG accumulation was absent in 19 of 24 patients. All six patients with Spigelman IV duodenal adenomas (without cancer) were negative; two of these underwent a duodenectomy and pathological examination did not reveal duodenal cancer. In five patients, FDG-PET revealed significant uptake, in the duodenum (2), lower abdomen (1), lung (1) and multiple sites in the abdomen (1). These hot spots correlated with duodenal cancer (2), abdominal metastasis (1) and sclerosing haemangioma of the lung (1). We failed to make a histopathological diagnosis in the single patient with multiple intra-abdominal sites of FDG uptake. None of the patients from the FDG-PET-negative group developed cancer during follow-up (mean 2.8 years). FDG-PET detected all the cancers present, and none of the patients with negative FDG-PET developed cancer. This suggests that positive FDG-PET in FAP patients should lead to further examinations to rule out cancer. In patients with negative FDG-PET a more conservative approach seems justified.
    European journal of nuclear medicine and molecular imaging 04/2006; 33(3):270-4. DOI:10.1007/s00259-005-1955-0
  • [Show abstract] [Hide abstract]
    ABSTRACT: We describe the case of an 18-year-old female with Cowden syndrome in whom a simple x-ray detected a solitary pulmonary nodule that was identified as a sclerosing hemangioma. Pulmonary sclerosing hemangioma is an unusual lung neoplasm which typically presents as a solitary peripheral nodule in asymptomatic women. Although the histology of this entity is well defined, its origin and treatment is debated. One of the main diagnostic problems is to histologically differentiate a pulmonary sclerosing hemangioma from a papillary lung carcinoma.Describimos el caso de una paciente de 18 años con síndrome de Cowden, en quien una radiografía simple objetivó un nódulo pulmonar solitario que resultó ser un hemangioma esclerosante. El hemangioma esclerosante de pulmón es una neoplasia pulmonar poco frecuente, que suele presentarse como un nódulo solitario periférico en mujeres asintomáticas. En la actualidad, aunque la histología del hemangioma esclerosante está bien definida, son objeto de discusión la histiogénesis de esta entidad y su tratamiento. Uno de los principales problemas diagnósticos es diferenciar histológicamente el hemangioma esclerosante del adenocarcinoma pulmonar con patrón papilar.
    Archivos de Bronconeumología 07/2007; 43(7-43):418-420. DOI:10.1016/S1579-2129(07)60094-9
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: We present a 53-year-old female suffering from familial adenomatous polyposis, who was found to have a positive nodus, lateral to the hilus of the left lung, on routine FDG-PET scan. This lesion was found to be a sclerosing hemangioma. We found an aberrant beta-catenin expression on immunohistochemical staining, suggesting that sclerosing hemangioma and familial adenomatous polyposis share the same pathophysiology. It is important to be aware of the association of familial adenomatous polyposis and sclerosing hemangioma.
    Diseases of the Colon & Rectum 12/2007; 50(11):1987-91. DOI:10.1007/s10350-006-0862-1