Ann Thorac Surg 2008;86:310-312. doi:10.1016/j.athoracsur.2007.10.016
© 2008 The Society of Thoracic Surgeons
Case Reports
Transdiaphragmatic Amyloidoma
Daniel J. Rosen, MDa,
Christine Stavropoulos, MDa,
William D. Travis, MDb,
Robert C. Ashton, MDc,
Faiz Y. Bhora, MDa,
Cliff P. Connery, MDa,*
a Department of Thoracic Surgery, St. Luke's-Roosevelt Hospital Center, New York, New York
b Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York
c Division of Thoracic Surgery, Hackensack University Medical Center, Hackensack, New Jersey
Accepted for publication October 2, 2007.
* Address correspondence to Dr Connery, Thoracic Surgery Division, St. Luke's—Roosevelt Hospital Center, 1000 10th Avenue, Suite 2B-07, New York, NY 10019 (Email: cconnery{at}chpnet.org).
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Abstract
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The term "amyloidoma" has been used to describe localized pulmonary nodular amyloidosis when it is a solitary lesion. Amyloidoma is an uncommon and infrequently reported cause of benign pulmonary lesions. We report the case of a 45-year-old man with hemoptysis, eosinophilia, and a large mass involving both lobes of the left lung, the chest wall, and, via extension through the diaphragm, the liver. Clinical suspicion of ecchinococcal cyst led to treatment via en bloc excision rather than attempting tissue biopsy for diagnosis. Complete resection of the isolated pulmonary amyloidoma was achieved with no evidence of recurrence.
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Introduction
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Localized or nodular pulmonary amyloidosis can involve a spectrum of lesions from a single large mass to multifocal disease. Seemingly unrelated to other systemic amyloidoses, these isolated lung lesions have an excellent prognosis. In the wider amyloidosis literature, hemoptysis is considered a symptom in only 2% of patients. The bleeding may be associated with amyloid infiltration of blood vessels or bronchial lesions [1, 2].
A 45-year-old man with progressively worsening hemoptysis came to the emergency room with expectoration of clots. He denied fever or night sweats and had no history of smoking or occupational exposure. He had recently returned from a trip to his homeland, Ecuador. Physical examination revealed severely diminished breath sounds at the base of the left lung, with no other remarkable findings. A computed tomography (CT) scan of the chest exhibited a 14 x 9 x 7-cm pleural-based cystic mass emerging from the left lower lobe (Fig 1). Of note was a second cystic component, likely contiguous with the pleural mass, extending into the abdomen and displacing the fundus of the stomach laterally (Fig 2). Laboratory values were positive for eosinophilia (10.9%; normal, 0 to 5.0%).
Although findings on the imaging studies were not characteristic, with the recent travel history, eosinophilia, and a cystic mass, the possibility of echinococcal cyst was considered in the differential diagnosis. Accordingly, further attempts at obtaining a tissue diagnosis via biopsy were not made, to prevent rupture of the cyst capsule. Because the patient had symptoms, a decision was made to proceed directly to surgical excision of the mass. Before resection of the mass, serologic tests for echinococcal organisms were performed. Despite negative results, the decision was made to treat prophylactically with albendazole because the sensitivity of serologic testing is only 56% [3, 4].
The mass was initially approached through a thoracotomy incision. At visualization, it had a gray, waxy, chronically scarred–appearing capsule. It was found to be densely adherent to the chest wall at its most lateral aspect. To prevent rupture of the cystic mass a rib resection was performed, with the segments of the two ribs dropping down within the thorax, enabling continued mobilization of the tumor. The entirety of the tumor was freed from the upper and lower lobes of the lung via deep wedge resection using the gastrointestinal anastomosis 75-mm staplers with "green load" 4.1-mm staples. The mass, now unattached in the pleural cavity, was noted to extend through the diaphragm. The thoracotomy incision was extended across to the upper part of the abdomen, transecting the costal cartilage to enable simultaneous thoracic and abdominal mobilization.
In the abdomen, the mass was adherent to the omentum and the left lobe of the liver. The omentum was divided between clamps and silk ligatures. The medial portion of the mass was freed from the liver by performing a small wedge excision of the lateral portion of the left lobe using a cautery. En bloc resection was completed by dividing the diaphragmatic fibers circumferentially around the 15-inch-long, pale tan, waxy mass, which was delivered in its entirety to the pathology department for analysis. Despite the mass being removed intact, the wound was copiously irrigated with hypertonic saline solution in the event of echinococcal seeding. Closure of the diaphragmatic defect was accomplished with bovine pericardium to avert undue tension, and an additional piece was used to reconstruct the chest wall defect.
Histopathologic analysis of the excised mass revealed a cavitary nodule with a thick capsule consisting of a dense fibrous wall. There was a marked histiocytic and multinucleated giant cell reaction, as well as hemosiderin deposition suggesting chronic hemorrhage. Within the capsule were two distinct cysts containing necrotic debris. The mass lesion had amorphous, eosinophilic, extracellular deposits with the unequivocal diagnostic features of amyloid. The eosinophilic material was also deposited within the vascular walls in adjacent lung parenchyma included with the specimen. These amorphous eosinophilic deposits showed apple-green birefringence at examination with Congo red staining and polarized light microscopy. Because the specimen was lymphocyte poor, immunohistochemistry was not performed. No parasitic organisms were seen.
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Comment
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Pulmonary amyloidosis is a common manifestation of primary amyloidosis. The amyloid deposits may be seen in tracheobronchial form or in diffusely interstitial or nodular patterns [5–7]. Solitary lesions, termed "localized nodular pulmonary amyloidosis" or "amyloidomas," are often not associated with any other systemic amyloidoses [8]. Patients with nodular disease usually have no pulmonary symptoms, though they may have bronchiectasis or hemoptysis from bronchiole compression or invasion. Those with interstitial disease more typically have dyspnea [9]. Often the only laboratory abnormality is eosinophilia and a parasitic mass such as Echinococcus, and hydatid cyst should remain in the differential diagnosis [10].
These isolated amyloidomas are typically slow growing and have been described up to 15 cm in greatest diameter. At gross examination these lesions have a firm, waxy, gray or pale tan capsule. Larger lesions are often found to cavitate, with focal areas of hemorrhage, necrosis, fibrosis, or calcification [4]. At microscopic examination the lesions exhibit an eosinophilic lymphoplasmacytic infiltrate with multinucleated giant cells. Under polarizing light microscopy amorphous amyloids demonstrate an apple-green birefringence. The amyloid often encases arteries and veins contiguous to the mass in adjacent lung tissue [3].
Growth is likely through invasion into adjacent tissue via local extension with necrosis. This growth makes these nodular amyloid masses difficult to differentiate clinically or radiographically from bronchogenic carcinoma. Although needle biopsy of amyloidomas via CT-guided percutaneous and transbronchial approaches has been described, it should be avoided because of the risks of hemorrhage, rupture of an unrecognized echinococcal cyst, and potential air embolus [11–15]. In addition, a normal needle biopsy result cannot definitively rule out malignancy. Therefore, excisional biopsy via thoracotomy or thoracoscopy offers the best opportunity to obtain both a definitive diagnosis and curative resection.
Because the differential diagnosis includes bronchogenic carcinoma and parasitic cyst, treatment is en bloc surgical excision. Prognosis after resection of isolated amyloidomas is excellent. Recurrence is rare [4]. Some have suggested observing asymptomatic small lesions in patients with known pulmonary amyloidosis [16].
In conclusion, we present a case of isolated amyloidoma, locally invasive with extension through the diaphragm into the abdominal cavity. Wide surgical excision of this large amyloidoma was curative. The patient has remained well, without CT evidence of recurrence at 30 months of follow-up.
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References
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- Yood RA, Skinner M, Rubinow A, Talarico L, Cohen AS. Bleeding manifestations in 100 patients with amyloidosis JAMA 1983;249:1332-1334.
- Tariq SM, Morrison D, McConnochie K. Solitary bronchial amyloid presenting with haemoptysis Eur Respir J 1990;3:1230-1231.[Abstract]
- Zhang W, McManus DP. Concepts of immunology and diagnosis of hydatid disease Clin Microbiol Rev 2003;16:18-36.[Abstract/Free Full Text]
- Ortona E, Riganò R, Butarri B, et al. An update on immunodiagnosis of cystic ecchinococosis Acta Trop 2003;85:165-171.[Medline]
- Gertz MA, Greipp PR. Clinical aspects of pulmonary amyloidosis Chest 1986;90:790-791.[Medline]
- Thompson PJ, Citron KM. Amyloid and the lower respiratory tract Thorax 1983;38:84-87.[Free Full Text]
- Hui AN, Koss MN, Hochholzer L, Wehunt WD. Amyloidosis presenting in the lower respiratory tract: clinicopathologic, radiographic, immunohistochemical, and histochemical studies on 48 cases Arch Pathol Lab Med 1986;110:212-218.[Medline]
- Leu CY, Lynch D, Chan E. The case of the torpid thoracic tumor Chest 1997;112:535-537.[Medline]
- Farell M, McAdams HP, Erasmus JJ. A 27-year-old woman with cough, dyspnea, and wheezing Chest 1999;116:557-559.[Medline]
- Haydar AA, Li A, Goldsmith DJA. Eosinophilia and symptomatic pulmonary amyloidosis Postgrad Med J 2004;80:738-739.[Abstract/Free Full Text]
- Kline LR, Dise CA, Ferro TJ, Hansen-Flaschen JH. Diagnosis of pulmonary amyloidosis by transbronchial biopsy Am Rev Respir Dis 1985;132:191-194.[Medline]
- Utz JP, Swenson SJ, Gertz MA. Pulmonary amyloidosis: the Mayo Clinic experience from 1980 to 1993 Ann Intern Med 1996;124:407-413.[Abstract/Free Full Text]
- Scott PP, Scott WW. Isolated nodular pulmonary amyloidosis: diagnosis by percutaneous needle aspiration biopsy South Med J 1985;78:467-471.[Medline]
- Dundore PA, Aisner SC, Seidman JD. Nodular pulmonary amyloidosis: diagnosis by fine-needle aspiration cytology and a review of the literature Diagn Cytopathol 1993;9:562-564.[Medline]
- Strange C, Heffner JE, Collins BS. Pulmonary hemorrhage and air embolism complicating transbronchial biopsy in pulmonary amyloidosis Chest 1987;92:367-369.[Medline]
- Cordier JF, Loire R, Brune J. Amyloidosis of the lower respiratory tract: clinical and pathologic features in a series of 21 patients Chest 1986;90:827-831.[Medline]
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Abstract
Introduction
