IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Lymphadenopathy and Upper Airway Obstruction Andrew Li1, Kay Leong Khoo1, Char Loo Tan2, Angela Pang3, and Pyng Lee1 1 Division of Respiratory and Critical Care Medicine and 2Department of Pathology, National University Hospital, Singapore; and 3Department of Medical Oncology, National Cancer Institute of Singapore, Singapore
Figure 1. Chest radiograph revealed the presence of a prominent left paratracheal stripe (yellow arrow) with an endotracheal tube present.
A middle-aged Chinese woman presented to the emergency department with stridor and acute type 2 respiratory failure. She had previously declined surgery for a multinodular goiter diagnosed on ultrasound. Flexible nasoendoscopy showed normal vocal cords and lateral tracheal bulge, presumably from extrinsic compression by the goiter. She was intubated (Figure 1) and underwent emergent total thyroidectomy to relieve the upper airway obstruction. Intraoperatively, a tumor with features of follicular dendritic cell sarcoma (FDCS) was found, displacing the thyroid and extending retrosternally (Figures 2A–2E). Postoperative 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed positron emission tomography-avid mediastinal lymph nodes (Figure 3), and the patient was referred for evaluation. Bronchoscopy incidentally showed a polypoidal tracheal tumor 4 cm below the vocal cords, causing 40% obstruction, which was resected with an electrosurgical snare. Tracheal biopsy revealed metastatic FDCS (Figures 4A–4C). Despite chemoradiotherapy, the patient presented 8 months later with near-total airway obstruction by the recurrent tracheal tumor, which necessitated rigid bronchoscopy, neodymium-YAG photoresection, and silicon stent placement (Figure 5). FDCS is a rare primary lymph node neoplasm arising from follicular dendritic reticulum, with only 100 cases reported. There is growing evidence to suggest a temporal relationship between FDCS and the hyaline-vascular type of Castleman’s disease from hyperplasia, dysplasia, and neoplasia, which is evident in our patient’s resected tumor (Figure 2C) (1, 2). Extranodal FDCS has been described in the thyroid, nasopharynx, liver, and retroperitoneum (3, 4) and is not often considered because of its rarity, leading to a misdiagnosis rate of 30% (5). Our unusual case is the first of FDCS masquerading as goiter with upper airway obstruction from presumed extrinsic compression, and subsequently revealing as secondary tracheal tumor. Endotracheal metastasis (6) can occur by invasion from surrounding structures (type 1), from parenchymal lesion (type 2), from mediastinal or hilar lymph nodes (type 3), or from a peripheral lesion that extended along the proximal bronchus (type 4). Tracheal biopsy demonstrating intact epithelium supported type 1 endotracheal metastasis. Surgical resection with adjuvant chemoradiotherapy is the recommended treatment (4), and 27.4% 5-year recurrence-free survival has been reported (5). n
Am J Respir Crit Care Med Vol 191, Iss 1, pp e1–e3, Jan 1, 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1164/rccm.201409-1622IM Internet address: www.atsjournals.org
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. (A) Photograph of the excised fleshy and myxoid pinkish tumor with areas of cystic degeneration and hemorrhage (green arrow). The tumor is compressing on and displacing normal thyroid tissue (red arrow), mimicking a multinodular goiter. (B) Histology section of tumor, showing transition between macroscopically fleshy (left) and myxoid (right) areas. Hematoxylin and eosin: magnification, 2003. (C) Low-power examination showing tumor (dotted outline) within the lymph node containing atretic follicles (yellow arrow), consistent with Castleman’s disease. Hematoxylin and eosin; magnification, 303. (D) Histology section of fleshy tumor showing scattered ovoid cells with prominent nucleoli (white arrow) in the background of lymphocytes. Hematoxylin and eosin; magnification, 4003. (E) Higher magnification of the atretic lymphoid follicle showing a penetrating hyalinized vessel in keeping with hyaline-vascular type Castleman’s disease. Hematoxylin and eosin; magnification, 1003.
Figure 3. Positron emission tomography/computed tomography showing fluoro-deoxyglucose-avid mediastinal lymph nodes with surrounding increase in uptake (green) from inflammation after thyroidectomy.
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American Journal of Respiratory and Critical Care Medicine Volume 191 Number 1 | January 1 2015
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Figure 4. (A) Tracheal biopsy showing ciliated bronchial epithelium (red arrow) overlying myxomatous tumor similar in morphology to primary follicular dendritic cell sarcoma, as depicted in Figure 2B. Hematoxylin and eosin; magnification, 2803. (B) Higher magnification showing scattered atypical ovoid cells. Hematoxylin and eosin; magnification, 4003. (C) The atypical ovoid cells stained positive for CD35, confirming metastatic follicular dendritic cell sarcoma in trachea. Magnification, 4003.
Figure 5. (A) Polypoidal tumor causing near-total obstruction of trachea. (B) Neodymium-YAG laser ablation of tumor with recanalization of trachea. (C) Silicone stent placement. Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Jiang L, Zhao LY, Liu Y, Zhao YF. Castleman’s disease of the neck: report of 4 cases with unusual presentations. J Oral Maxillofac Surg 2011;69:1094–1099. 2. Wang H, Su Z, Hu Z, Wen J, Liu B. Follicular dendritic cell sarcoma: a report of six cases and a review of the Chinese literature. Diagn Pathol 2010;5:67–72. 3. Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, Hagemeister FB, Pro B. Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature. Am J Hematol 2007;82:725–728.
4. Kairouz S, Hashash J, Kabbara W, McHayleh W, Tabbara IA. Dendritic cell neoplasms: an overview. Am J Hematol 2007;82: 924–928. 5. Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, Nobrega J, Wong WD, Klimstra DS. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Arch 2006;449: 148–158. 6. Kiryu T, Hoshi H, Matsui E, Iwata H, Kokubo M, Shimokawa K, Kawaguchi S. Endotracheal/endobronchial metastases: clinicopathologic study with special reference to developmental modes. Chest 2001;119:768–775.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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Figure 1. Chest radiograph revealed the presence of a prominent left paratracheal stripe (yellow arrow) with an endotracheal tube present.
A middle-aged Chinese woman presented to the emergency department with stridor and acute type 2 respiratory failure. She had previously declined surgery for a multinodular goiter diagnosed on ultrasound. Flexible nasoendoscopy showed normal vocal cords and lateral tracheal bulge, presumably from extrinsic compression by the goiter. She was intubated (Figure 1) and underwent emergent total thyroidectomy to relieve the upper airway obstruction. Intraoperatively, a tumor with features of follicular dendritic cell sarcoma (FDCS) was found, displacing the thyroid and extending retrosternally (Figures 2A–2E). Postoperative 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed positron emission tomography-avid mediastinal lymph nodes (Figure 3), and the patient was referred for evaluation. Bronchoscopy incidentally showed a polypoidal tracheal tumor 4 cm below the vocal cords, causing 40% obstruction, which was resected with an electrosurgical snare. Tracheal biopsy revealed metastatic FDCS (Figures 4A–4C). Despite chemoradiotherapy, the patient presented 8 months later with near-total airway obstruction by the recurrent tracheal tumor, which necessitated rigid bronchoscopy, neodymium-YAG photoresection, and silicon stent placement (Figure 5). FDCS is a rare primary lymph node neoplasm arising from follicular dendritic reticulum, with only 100 cases reported. There is growing evidence to suggest a temporal relationship between FDCS and the hyaline-vascular type of Castleman’s disease from hyperplasia, dysplasia, and neoplasia, which is evident in our patient’s resected tumor (Figure 2C) (1, 2). Extranodal FDCS has been described in the thyroid, nasopharynx, liver, and retroperitoneum (3, 4) and is not often considered because of its rarity, leading to a misdiagnosis rate of 30% (5). Our unusual case is the first of FDCS masquerading as goiter with upper airway obstruction from presumed extrinsic compression, and subsequently revealing as secondary tracheal tumor. Endotracheal metastasis (6) can occur by invasion from surrounding structures (type 1), from parenchymal lesion (type 2), from mediastinal or hilar lymph nodes (type 3), or from a peripheral lesion that extended along the proximal bronchus (type 4). Tracheal biopsy demonstrating intact epithelium supported type 1 endotracheal metastasis. Surgical resection with adjuvant chemoradiotherapy is the recommended treatment (4), and 27.4% 5-year recurrence-free survival has been reported (5). n
Am J Respir Crit Care Med Vol 191, Iss 1, pp e1–e3, Jan 1, 2015 Copyright © 2015 by the American Thoracic Society DOI: 10.1164/rccm.201409-1622IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 2. (A) Photograph of the excised fleshy and myxoid pinkish tumor with areas of cystic degeneration and hemorrhage (green arrow). The tumor is compressing on and displacing normal thyroid tissue (red arrow), mimicking a multinodular goiter. (B) Histology section of tumor, showing transition between macroscopically fleshy (left) and myxoid (right) areas. Hematoxylin and eosin: magnification, 2003. (C) Low-power examination showing tumor (dotted outline) within the lymph node containing atretic follicles (yellow arrow), consistent with Castleman’s disease. Hematoxylin and eosin; magnification, 303. (D) Histology section of fleshy tumor showing scattered ovoid cells with prominent nucleoli (white arrow) in the background of lymphocytes. Hematoxylin and eosin; magnification, 4003. (E) Higher magnification of the atretic lymphoid follicle showing a penetrating hyalinized vessel in keeping with hyaline-vascular type Castleman’s disease. Hematoxylin and eosin; magnification, 1003.
Figure 3. Positron emission tomography/computed tomography showing fluoro-deoxyglucose-avid mediastinal lymph nodes with surrounding increase in uptake (green) from inflammation after thyroidectomy.
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American Journal of Respiratory and Critical Care Medicine Volume 191 Number 1 | January 1 2015
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 4. (A) Tracheal biopsy showing ciliated bronchial epithelium (red arrow) overlying myxomatous tumor similar in morphology to primary follicular dendritic cell sarcoma, as depicted in Figure 2B. Hematoxylin and eosin; magnification, 2803. (B) Higher magnification showing scattered atypical ovoid cells. Hematoxylin and eosin; magnification, 4003. (C) The atypical ovoid cells stained positive for CD35, confirming metastatic follicular dendritic cell sarcoma in trachea. Magnification, 4003.
Figure 5. (A) Polypoidal tumor causing near-total obstruction of trachea. (B) Neodymium-YAG laser ablation of tumor with recanalization of trachea. (C) Silicone stent placement. Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Jiang L, Zhao LY, Liu Y, Zhao YF. Castleman’s disease of the neck: report of 4 cases with unusual presentations. J Oral Maxillofac Surg 2011;69:1094–1099. 2. Wang H, Su Z, Hu Z, Wen J, Liu B. Follicular dendritic cell sarcoma: a report of six cases and a review of the Chinese literature. Diagn Pathol 2010;5:67–72. 3. Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, Hagemeister FB, Pro B. Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature. Am J Hematol 2007;82:725–728.
4. Kairouz S, Hashash J, Kabbara W, McHayleh W, Tabbara IA. Dendritic cell neoplasms: an overview. Am J Hematol 2007;82: 924–928. 5. Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, Nobrega J, Wong WD, Klimstra DS. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Arch 2006;449: 148–158. 6. Kiryu T, Hoshi H, Matsui E, Iwata H, Kokubo M, Shimokawa K, Kawaguchi S. Endotracheal/endobronchial metastases: clinicopathologic study with special reference to developmental modes. Chest 2001;119:768–775.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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