|Year : 2014 | Volume
| Issue : 1 | Page : 13-14
Pulmonary hyalinizing granuloma involving the diaphragm and the pericardium
Haranahally Raghavan Vanisri1, Satish Suchitha2, Hungund Chandrakanth2, Gubanna Vimalambika Manjunath1
1 Department of Pathology, JSS Medical College, JSS University, Mysore, Karnataka, India
2 Department of Forensic Medicine, JSS Medical College, JSS University, Mysore, Karnataka, India
|Date of Web Publication||28-Jul-2014|
Haranahally Raghavan Vanisri
Department of Pathology, JSS Medical College, JSS University, Mysore, Karnataka
Source of Support: None, Conflict of Interest: None
Pulmonary hyalinizing granuloma (PHG) is a rare disorder and is a pertinent differential for lung diseases with multiple pulmonary nodules. Natural history of this disease is not known. Although the usual course is benign, a close follow-up of these cases is necessary. We herein report a case of PHG involving the diaphragm and the heart, detected on autopsy in a 57-year-old male who suffered a road traffic accident.
Keywords: Diaphragmatic nodules, pericardium, pulmonary hyalinizing granuloma
|How to cite this article:|
Vanisri HR, Suchitha S, Chandrakanth H, Manjunath GV. Pulmonary hyalinizing granuloma involving the diaphragm and the pericardium. Int J Stud Res 2014;4:13-4
|How to cite this URL:|
Vanisri HR, Suchitha S, Chandrakanth H, Manjunath GV. Pulmonary hyalinizing granuloma involving the diaphragm and the pericardium. Int J Stud Res [serial online] 2014 [cited 2019 Apr 26];4:13-4. Available from: http://www.ijsronline.net/text.asp?2014/4/1/13/137615
| Introduction|| |
Pulmonary hyalinizing granuloma (PHG) is a rare disease with distinct fibrosing lesions of the lung characterized by central whorled deposits of lamellar collagen. It has been reported that PHG is accompanied by extra-pulmonary fibrous lesions at various sites including the kidney, tonsils and thyroid glands. PHG presents as pulmonary nodules with non specific symptoms of cough, hemoptysis, chest pain and shortness of breath. When asymptomatic, it is usually detected on routine chest radiograph. An immune response to the antigenic stimuli by infection or autoimmune process has been postulated in the pathogenesis but the precise etiology remains obscure . The lesion can be situated in the lung parenchyma or sub pleura. Due to their behavior, a biopsy is required to establish the primary diagnosis of PHG . To the best of our knowledge, this is the first encounter with a case of pulmonary hyalinizing granuloma with involvement of the pleural surface of the diaphragm and heart without involvement of the lung parenchyma.
| Case Report|| |
A medico legal autopsy was performed at our institute on a 57-year-old male who died following a road traffic accident. History obtained from his relatives and medical records revealed him to be a chronic smoker for past 30 years and a known hypertensive since 3 years, and on single drug antihypertensive therapy. There was no history suggestive of any infection or autoimmune disease. Autopsy revealed multiple, bilateral, well-circumscribed, rubbery white nodules on the diaphragm, largest measuring 3 × 4 cm and the smallest 1 × 1 cm. There was no pulmonary involvement [Figure 1]a] on gross appearance. In addition to the diaphragmatic nodules, careful examination of the pericardium also showed presence of two such similar nodules [Figure 1]b]. Histopathological examination of these individual nodules showed bundles of lamellar hyalinized collagen arranged in parallel and whorl configuration [Figure 2]a], admixed perivascular lymphoplasmacytic infiltration was noted [Figure 2]b]. There was no evidence of granulomas or areas of necrosis in the multiple sections that were studied.
|Figure 1 (a) Bilateral irregular multiple white nodules on the diaphragm with normal lung below (b) Irregular white nodules on the pericardial surface of the heart|
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|Figure 2 (a) Homogenous hyalinized lamellae of collagen arranged in parallel formation (H and E, ×100) (b) Perivascular lymphoplasmacytic aggregates around hyalinized collagen bands (H and E, ×200)|
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Masons' trichrome stain and van Gieson's stain were done, which confirmed the presence of collagen. The nodular section was also subjected to special stains like acid fast and Congo red to rule out mycobacterial involvement and amyloidosis respectively. A final diagnosis of PHG was made that invariably involved the diaphragm and the pericardium.
| Discussion|| |
PHG is a rare benign condition first described in 1977, which usually manifests as multiple bilateral pulmonary nodules of lamellar hyaline collagen deposits. It usually affects people of age 19-77 years with a mean age of 44 years at the time of presentation and has no gender predilection . Size of the tumor varies from several millimeters to 15 cm in greatest dimensions and 73% of such patients have multiple lesions . Majority of the patients are asymptomatic, which correlated with the present case.
The etiology of PHG is unknown, but it has been associated with immunologic or infectious diseases such as rheumatoid arthritis, sclerosing mediastinitis, retroperitoneal fibrosis, uveitis, occulopapillitis, tuberculosis, histoplasmosis and aspergillosis . Neoplastic diseases have rarely been reported which include abdominal lymphoma, multiple myeloma, Paget's disease of breast and astrocytoma . The present case did not have any previous history of autoimmune or infective diseases or precisely, no medical records were available for the same. The lesion can be situated in the lung parenchyma or subpleura . PHG is sometimes accompanied by extra-pulmonary fibrous lesions at other sites, and coexistence of PHG along with laryngeal and subcutaneous nodules have been reported . The present case did not show extra pulmonary fibrous lesions at other sites.
The disease follows a relatively benign course with the nodules showing increasing in size over a period of years. There are two reported cases of PHG complicated by lymphoma. Therefore, a follow-up is utmost essential in such cases . Prognosis of PHG is excellent with no significant impact on longevity . There have been reports of patients who responded well to corticosteroid therapy . Surgical resection is the treatment of choice .
| Conclusion|| |
Diagnosis of PHG requires a histological examination, and therefore, a biopsy is essential. In previously reported cases, most lesions occurred in the lung with extra-pulmonary manifestations. This is a rare case of PHG involving the diaphragm and the heart without involvement of the lung, discovered incidentally at autopsy.
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