Primary Pulmonary Amyloidosis with Mediastinal LymphadenopathyDohun Kim, M.D.

ISSN: 2233-601X (Print) ISSN: 2093-6516 (Online)

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Departments of

Thoracic and Cardiovascular Surgery and

Pathology, Chungbuk National University College of Medicine Received: July 24, 2015, Revised: August 22, 2015, Accepted: August 25, 2015, Published online: June 5, 2016

Corresponding author: Jong-Myeon Hong, Department of Thoracic and Cardiovascular Surgery, Chungbuk National University College of Medicine, 1 Chungdae-ro, Seowon-gu, Cheongju 28644, Korea

(Tel) 82-43-269-6062 (Fax) 82-43-269-6969 (E-mail) [email protected]

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The Korean Society for Thoracic and Cardiovascular Surgery. 2016. All right reserved.

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This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creative- commons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Primary Pulmonary Amyloidosis with Mediastinal Lymphadenopathy

Dohun Kim, M.D.

, Yong-Moon Lee, M.D.

, Si-Wook Kim, M.D.

, Jong-Won Kim, M.D.

, Jong-Myeon Hong, M.D., Ph.D.

We report a case of inadvertent hoarseness after surgery for primary pulmonary amyloidosis. A 55-year-old male was transferred to our facility due to a lung mass. Chest computed tomography revealed a solitary pulmonary nodule. Positron emission tomography –computed tomography showed fluorodeoxyglucose uptake in the main mass and in the mediastinal lymph nodes. To confirm the pathology of the mass, wedge resection and thorough lymph node dissection were performed via video-assisted thoracic surgery (VATS). No complications except for hoarseness were observed; hoarseness developed soon after surgery and lasted for 3 months. The main mass was diagnosed as amyloidosis, but this was not found in the lymph nodes. In conclusion, VATS wedge resection for peripheral amyloidosis is a feasible and safe procedure. However, mediastinal lymph node dissection is not recommended un- less there is evidence of a clear benefit.

Key words: 1. Solitary pulmonary nodule 2. Amyloidosis

3. Video-assisted thoracic surgery

CASE REPORT

Primary pulmonary amyloidosis is a rare disease that can be mistaken for lung cancer [1]. Although percutaneous lung biopsy and other diagnostic modalities including positron emission tomography–computed tomography (PET-CT) can be helpful, surgical resection is required to determine the exact pathologic diagnosis [2]. Subaortic lymph nodes in particular are difficult to investigate without surgery. We herein report a case of primary pulmonary amyloidosis with mediastinal lymphadenopathy and inadvertent postoperative complications.

A 55-year-old man was transferred to the department of thoracic surgery due to a solitary pulmonary nodule in the left lower lobe (Fig. 1A). The nodule was found 3 months prior and had increased in size on follow-up chest CT, imply-

ing lung cancer. PET-CT showed subtle fluorodeoxyglucose uptake in the pulmonary nodule, but strong uptake in the me- diastinal lymph node (Fig. 1B). A percutaneous lung biopsy was performed, but the results were insufficient for diagnosis.

Therefore, video-assisted thoracic surgery (VATS) was performed. The pulmonary nodule was located in a major fis- sure and was found easily. Wedge resection was performed and the cut edge of the mass showed grayish tan-colored, firm features. The subaortic lymph node was located deep un- der the aorta, and careful dissection was performed to avoid injury to the vagus or recurrent laryngeal nerve (Fig. 2).

Electrocautery was also limited in order to avoid thermal injury. Lymph node sampling was attempted, but thorough dissection was unavoidable because massive bleeding oc- curred along the cut edge of the lymph node. There were no

Korean J Thorac Cardiovasc Surg 2016;49:218-220 _□ Case Report _□

http://dx.doi.org/10.5090/kjtcs.2016.49.3.218

Unnecessary Lymph Node Dissection in Pulmonary Amyloidoma

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Fig. 1. Chest CT and PET-CT of the main mass (*) and mediastinal lymph nodes (!). (A) Solitary pulmonary nodule in the left lower lobe on chest CT. (B) Pulmonary nodule and mediastinal lymphadenopathy on PET-CT. CT, computed tomography; PET, positron emission tomography.

Fig. 2. Operative view for subaortic lymph node dissection. The subaortic lymph node was dissected completely.

postoperative complications, with the exception of hoarseness.

Although vocal symptoms improved upon follow-up in the outpatient clinic, vocal cord palsy was still observed on post- operative day 90. In pathologic reports, amorphous eosino- philia and positive Congo red staining were found in the pul- monary nodule, consistent with amyloidosis (Fig. 3). Lymph involvement of amyloidosis was not found, but reactive hy- perplasia was observed in all mediastinal lymph nodes.

Further diagnostic tests to rule out systemic diseases that could lead to secondary amyloidosis (e.g., multiple myeloma) were performed. All blood tests with light chain lambda, kap-

pa, beta 2-microglobulin, and protein electrophoresis in serum and urine were within normal limits. However, a brain mag- netic resonance imaging was not performed due to patient refusal. The nodule was diagnosed as primary pulmonary amyloidosis, and regular follow-up was planned without addi- tional treatment.

DISCUSSION

Primary pulmonary amyloidosis is a rare disease charac- terized by the deposition of immunoglobulin light-chain frag- ments [3]. Intrathoracic manifestations are broad, including tracheobronchial, parenchymal, and pleural involvement in ad- dition to mediastinal lymphadenopathy [2,4]. Pulmonary amy- loidosis can be associated with lymphoproliferative diseases such as mucosa-associated lymphoid tissue lymphoma or mul- tiple myeloma [5]. Percutaneous lung biopsy is typically suf- ficient for pathological diagnosis [1,6], but surgical resection is recommended when malignancy is suspected [2,7].

Excision of the lesion is the treatment of choice due to its

rare recurrence [4]. However, it remains unclear whether

lymph node dissection is necessary. According to this report,

lymph node dissection may be harmful in primary pulmonary

Dohun Kim, et al

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Fig. 3. Amorphous eosinophilia and positive Congo red staining were found in the pulmonary nodule. (A) H&E stain, ×400. (B) Congo red stain, ×400.

amyloidosis. Mediastinal lymphadenopathy is not a rare con- dition in the disease entity, but mediastinal involvement by amyloidosis is rare [8]. Careful subaortic lymph node dis- section was performed, but it resulted in hoarseness lasting for 3 months. Moreover, there was no pathologic evidence that the lymph nodes were involved in amyloidosis. Therefore, mediastinal sampling or biopsy is recommended over thorough dissection in the case of primary pulmonary amyloidosis.

In conclusion, treating peripheral amyloidosis with VATS wedge resection can be an effective option, but mediastinal lymph node dissection should be done carefully.

CONFLICT OF INTEREST

No potential conflict of interest relevant to this article was reported.

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