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Department of Medicine*, Surgery,** Pathology*** andRadiodiagnosis****, All India Institute of Medical Sciences, AnsariNagar, New Delhi - 110 029. IndiaReceived : 24.10.2002; Accepted : 21.3.2003

Case Report

Pulmonary Hyalinizing Granuloma Presenting withDysphagiaGC Khilnani,* A Kumar,** S Datta Gupta,*** A Surendranath*, S Sharma****

AbstractWe describe a middle aged, non-smoking female who presented with dysphagia and underwent repeatedendoscopies and oesophageal dilatation for a period of six months without any response. On imaging shewas found to be having a lobulated mass with a radiological differential diagnosis of malignancy, lymphomaor a rare inflammatory lesion. After an inconclusive CT guided biopsy the patient underwent thoracoscopyon which an unresectable mass was found. The biopsy from the mass revealed pulmonary hyalinizinggranuloma (PHG). To best of our knowledge this is the first case of PHG presenting as dysphagia reportedin the English Literature. Literature on this rare entity is reviewed.

was no history of cough, haemoptysis, dyspnoea, fatigue,loss of appetite, or arthralgia. She did not give history ofheartburn, sour eructation’s, water brash, haematemesis,malena, abdominal pain or diarrhoea. In the past there washistory of having received antituberculosis treatment ofgenital tuberculosis 15 years back. She also underwenthysterectomy with bilateral salpingo-oophorectomy foruterine fibroid and left ovarian cyst one year prior topresenting to us. Physical examination revealed a middle-aged female of thin built who was not dyspnoeic at rest. Shewas afebrile, with a pulse rate of 84/min, respiratory rate of18/min and blood pressure of 120/76 mm Hg. There was nopulsus paradoxus. There was no anaemia, icterus, cyanosis,clubbing, oedema feet and jugular venous pressure was notraised. Examination of respiratory system was unremarkable.She had undergone serial endoscopic oesophageal dilatationsbefore and after presenting to this hospital.

INVESTIGATION

On investigation Hb was 11.2 gm/dl. TLC was 4500/cumm and DLC was N66, L36, E3, M1, ESR was 35 mm/1sthr (Wintrobe). Fasting blood sugar was 85 mg/dl. Liverfunctions and renal functions were within normal limits. Thechest radiograph revealed homogenous opacities in rightlower zone and right hilum, and cardiac size was withinnormal limits. Pulmonary functions revealed vital capacityof 63.46% predicted and FEV1/FVC of 93%. Sputum forAFB and cytology done three times each were negative.Mantoux test was also negative. An echocardiogram waswithin normal limits. On barium swallow (Fig. 1) thoracicesophagus below the level of carina showed smooth, longsegment of concentric narrowing and persistent non-distensibility. Proximal esophagus showed hold-up of barium

INTRODUCTION

Patients complaining of dysphagia usually present togastroenterologist for management. In the absence of

pathology of oesophagus, a mediastinal or pulmonarypathology causing extrinsic compression of oesophagusshould be considered. Pulmonary hyalinizing granuloma(PHG), a term which is described as a rare histopathologicentity of unknown etiology, presents as pulmonary noduleswith minimal symptoms.1 The disease may present withoutany symptoms and may be detected on routine chestradiograph or may have symptoms such as cough,haemoptysis, chest pain, shortness of breath. Predominantfeatures are multiple pulmonary nodules consisting ofextracellular, eosinophilic hyaline lamellae. An immuneresponse to the antigenic stimuli by infection or autoimmuneprocess has been postulated in the pathogenesis but theprecise etiology yet remains obscure.2 This case is beingpresented for its unique presentation, rarity and highlightsthe importance of invasive techniques for achievingdiagnosis.

CASE HISTORY

A forty year old, married female, a non-smoker, presentedto us with a history of difficulty in swallowing for solids ofpast three years. She also gave a history of low gradeundocumented fever with evening rise for the same duration.She had lost 8 kgs of body weight during this period. There

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Fig. 1 : Ba swallow

Fig. 2 : CT scan of chest

and dilatation. Esophageal mucosal folds were normal. Thedistal esophagus and gastroesophageal junction wereunremarkable. The rheumatoid factor, antinuclear antibodyand, antinuclear cytoplasmic antibody (cANCA) werenegative. Serum C3 was 0.83 g/L (0.07 - 0.12 g)/Serum IgGwas 1.482 g/L (0.9 - 1.96 g/L), IgA 0.30 g/L (0.12 - 0.38/L)and IgM was 0.13 g/L (0.09 - 0.24 g/L). Uppergastrointestinal endoscopy revealed stricture at 30 cm andoesophagus was dilated to 12.8 mm. The scope could not be

negotiated into stomach and oesophageal biopsyrevealed pseudoepitheliomatous hyperplasia andchronic inflammation. A computed tomogram(CT) (Fig. 2) of chest revealed a lobulated softdensity mass of 8 X 7 X 5 cm in sizepredominantly involving the apical segment ofright lower lobe and extending laterally. Themass showed small calcific foci within it andhomogenous enhancement following contrastadministration with no areas of necrosis. It alsoshowed encasement of bronchus intermedius andthe proximal basal segmental bronchi. It wasalso seen to extend into the mediastinum causingoesophageal involvement in the form of markedmural thickening causing luminal compromise(Fig. 2). CT guided FNAC from the lung massrevealed only blood. On bronchoscopy there wasno endobronchial growth and bronchial biopsyshowed fibrocollagenous tissue with someinflammatory cells. Bronchial aspirate did notshow malignant cells and stain for acid fast bacilliwas negative. Patient was taken up forthoracoscopic lung biopsy. At surgery there wereextensive adhesions between the parietal and

visceral pleura. After mobilization ofadhesions, an intraparenchymal nodulewas felt in the right lower lobe, which waswedged out. Rest of the lung was normal.A biopsy was taken

On histopathology, multiple sectionsfrom the lung showed areas ofhyalinization. These areas were composedof thick collagenous bands which werearranged concentrically around bloodvessels in many areas. Numerouslymphocytes, histiocytes and plasma cellswere found either admixed in collagenousbands or focally as aggregates. Thesefeatures are diagnostic of pulmonaryhyalinizing granuloma (Fig. 3). Post-operative period was uneventful. Onfollow-up over the three years patient iskeeping in good health and has minimaldifficulty in swallowing. Repeat CT scanshave not shown increase in the size of lungmass.

DISCUSSION

Pulmonary hyalinizing granuloma is a rare pathology andpresents most commonly as slowly enlarging nodules in lungparenchyma. It has been reported in the literature as a formof localized amyloidosis, a granulomatous reaction,secondary to pulmonary histoplasmosis, an isolatedpulmonary hypersensitivity reaction, a plasma cell granulomaand sarcoidosis.3 These nodules are usually bilateral, andmay occasionally cavitate or calcify.4 However, PHG has

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also presented as sclerosing mediastinitis.5,6 The differentialdiagnosis chiefly includes neoplasms, rheumatoid nodules,macronodular variant of sarcoidosis, Wegenergranulomatosis, plasma cell granulomas, lymphomatoidgranulomatosis, histoplasmosis and other fungal infections.The cause of PHG is unknown but immune mechanism issuggested, as there are many immune-mediated diseases,which are associated. These include rheumatoid arthritis,sclerosing mediastinitis, retroperitoneal fibrosis, uveitis, andoculopapillitis.5 It is postulated that the disease is the resultof immune mechanism triggered by tubercular bacilli,histoplasma organisms or other infectious agents.5 It is alsosuggested that pulmonary lesions of differing etiologies mayterminate as hyalinizing granulomas. Engleman andassociates postulated that PHG represents a chronic immuneresponse to unidentified agents.1

PHG is usually found in young and middle-aged adultswho may be asymptomatic or present with mild symptomslike cough, fatigue, vague thoracic pain, fever, dyspnoea orhemoptysis.5 Our patient underwent several esophagealdilatations before getting x-ray chest done. Also, she hadsignificant constitutional symptoms. There are no reportsof PHG presenting with dysphagia.

The chest radiograph usually reveals multiple, solid, wellcircumscribed pulmonary nodules, which show ill-definedborders with or without calcification or necrosis within them.Our patient had a lung mass, which had calcification withinit. Usually they do not grow or grow slowly. A doublingtime of one year was reported in one case.5 These nodules

histologically are composed ofrandomly arranged bundles ofcollagen surrounded by chronicinflammatory cells. The diseasefollows a relatively benign coursewith the nodules slowlyincreasing in size over a periodof years.5 However, there are tworeported cases of PHGcomplicated by lymphoma,5

therefore, a follow up is required.Because of absence of markers abiopsy is required to establish theprimary diagnosis of PHG.5

The present case is reportedwith the intention of highlightingthe association of PHG withdysphagia, and mass stimulatingmalignancy. Hence in any caseof dysphagia with absence ofmucosal lesion, investigationsshould be directed to look forpulmonary or mediastinalpathology. This patient received

Fig. 3 : Histopathology of lung lesion

several oesophageal dilatations before the diagnosis of a massin the lung was made. We would agree that this entity wasnot considered in our differential diagnosis, which could onlybe achieved by a lung biopsy. The importance of computedtomogram in the diagnosis of PHG cannot be over-emphasized. A high index of suspicion with use of lungbiopsy would achieve diagnosis in such cases. Because ofrarity the natural history of this rare disease is not known.Although the usual course is benign a close follow up ofthese cases is warranted.

REFERENCES

1. Engleman P, Liebow AA, Gmelich J, et al. Pulmonary hyalinizinggranuloma. Am Rev Respir Dis 1977; 115:997-1008.

2. Atagi S, Sakatani M, Akira M, Yamamoto S, Ueda E. Pulmonaryhyalinizing granuloma with Castleman’s disease. Intern Med1994; 33:689-91.

3. Maijub AG, Giltman LI, Verner JL, Peace RJ. Pulmonaryhyalinizing granuloma. Ann Allergy 1985; 54:227-9.

4. Patel Y, Ishikawa S, MacDonnell KF. Pulmonary hyalinizinggranuloma presenting as multiple cavitary calcified nodules.Chest 1991; 100:1720-1.

5. Ren Y, Raitz EN, Lee KR, Pingleton SK, Tawfik O. Pulmonarysmall lymphocytic lymphoma (mucosa-associated lymphoidtissue type) associated with pulmonary hyalinizing granuloma.Chest 2001; 120:1027-30.

6. Kuramochi S, Kawai T, Yakumaru K, et al. Multiple pulmonaryhyalinizing granulomas associated with systemic idiopathicfibrosis. Acta Pathol Jpn 1991; 41:375-82.