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Heart, Lung and Circulation (2015) xx, 1–4 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.02.013

Successful Giant Thymic Cyst Removal: Case Report and Review of the Literature Scott Jennings, MBBS Hons a*, Robert G. Stuklis, FRACS b, Justin Chan, FRACS c, Daniel Kearney, FRCPA d Cardiothoracic Surgery Trainee, D’Arcy Sutherland Cardiothoracic Surgical Unit, Royal Adelaide Hospital[2_TD$IF], Adelaide, South Australia Consultant Cardiothoracic Surgeon, D’Arcy Sutherland Cardiothoracic Surgical Unit, Royal Adelaide Hospital[2_TD$IF], Adelaide, South Australia Cardiothoracic Surgery Fellow, D’Arcy Sutherland Cardiothoracic Surgical Unit, Royal Adelaide Hospital[2_TD$IF], Adelaide, South Australia d Anatomical [4_TD$IF]Pathologist, SA Pathology, Royal Adelaide Hospital[2_TD$IF], Adelaide, South Australia a

b c

Received 27 November 2014; received in revised form 6 February 2015; accepted 11 February 2015; online published-ahead-of-print xxx

Giant thymic cysts are a rare clinical entity evolving from smaller benign thymic cysts over many years. Benign thymic cysts account for approximately 3% of all mediastinal masses. There is a paucity of literature regarding benign thymic cyst management, especially when dealing with giant cysts. This can lead to potential confusion amongst clinicians[5_TD$IF] on how to best treat these patients. We report the successful diagnosis and treatment of a 76 year-old female with a giant, benign thymic cyst. This cyst was discovered incidentally and after consultation of the literature it was found management strategies regarding this condition are scarce. After careful consideration of surgical principles, patient preference and potential complications of a conservative approach, the successful surgical removal of a 1.8 kg cyst took place. The patient improved symptomatically with improved exercise tolerance and lung function tests. This case demonstrates the benefits of giant thymic cyst removal thus confirming diagnosis, reducing potential serious complications and improving patient quality of life. [6_TD$IF]Keywords

Benign thymic cyst  Median sternotomy  Giant thymic cyst  Excision

Introduction Congenital mediastinal cysts represent 3-6% of all mediastinal tumours and 10-18% of all mediastinal masses reported radiologically [1,2]. Benign thymic cysts are less common accounting for approximately 3% of all mediastinal masses [3]. Benign thymic cysts, are usually unilocular, contain clear fluid and are thin-walled. Benign thymic cysts are usually asymptomatic and are of little clinical importance being classified as an intra-thoracic mesothelial cyst, a congenital abnormality [1]. Embryologically the thymus is derived from the 3rd pharyngeal pouches with the ventral portions migrating caudally into the mediastinum. The non-neoplastic and leading theory postulates formation from the remnants of the thymopharyngeal ducts. The thymopharyngeal ducts undergoing cystic degeneration of ductal Hassall corpuscles then form benign thymic cysts [3,4].

Congenital cysts may be present throughout the thorax with posterior mediastinal cysts in particular having associated vertebral dislodgement and neurological symptoms [1,5]. It is critical that when these cysts enlarge, compressive effects are demonstrated and patients develop symptoms. Common symptoms on presentation of enlarged benign thymic cysts and mediastinal cysts in general include chest pain, dyspnoea, orthopnoea, wheeze, cough, fever and dysphonia [2]. It must be noted that benign thymic cysts can also co-exist with malignant tumours [6]. There is currently no agreed management practice when treating both asymptomatic and symptomatic patients with benign thymic cysts. Expectant management with surveillance imaging (watch and wait) is the most common primary approach. Strategies of both fine needle and mediastinoscopy aspiration and biopsy with diagnostic and curative intent have also been reported with varied results [3,7]. The following

*Corresponding author at: Cardiothoracic Surgery Trainee, D’Arcy Sutherland Cardiothoracic Surgical Unit, Royal Adelaide Hospital, South Australia. Tel.: +61 45 843 8864, Email: [email protected] © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.

Please cite this article in press as: Jennings S, et al. Successful Giant Thymic Cyst Removal: Case report and review of the literature. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.013

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presents the case of a giant thymic benign cyst and successful removal thereof. We hope that the sharing of the successful outcome in this case will aid fellow clinician’s decision-making when presented with patients with benign thymic cysts.

Case Report A 76 year-old female was referred after incidental finding of an anterior mediastinal mass. The patient was normally well with the only history of note being controlled hypertension and melanoma excision 30 years prior. The patient initially presented after suffering a minor trauma to her chest with a PA chest X-ray with what appeared to be an enlarged, globular heart (Figure 1a). Prompt CT scan of the chest revealed a large, anterior mediastinal, fluid-filled, well-demarcated cyst. The cyst extended from the superior mediastinum caudally on either side of the heart with maximum dimensions transversely of 20.5 cm and 16.2 cm cranio-caudally (Figure 2). The anterior-posterior component measured 10 cm. There

were no solid components or septations noted. Compressive atelectasis was seen in the right middle and lower lobes as well as the lingular on the left. The remainder of the scan was unremarkable. Radiologically, the provisional diagnosis was of giant mediastinal benign thymic cyst. On examination, the patient was not dyspnoeic with physical examination unremarkable. The patient denied any red flag symptoms and felt generally well. However, on specific questioning, recent orthopnoea and dyspnoea on exertion was reported with a productive morning cough. It was felt the best to remove the cyst en bloc via median sternotomy. Despite being asymptomatic, it was feared that complications associated with large thymic cysts may supervene if a conservative approach was taken. Lung function testing results: FEV1 of 1.45L and FVC of 1.94L, both 75% of predicted. Corrected DLCO was 12.4 (64% of predicted) with Arterial Blood Gas and routine bloods normal. Median sternotomy was performed with successful removal of the intact cyst (Figure 3). Histological examination revealed

Figure 1 Pre- (1a) and post-operative (1b) Chest X-rays.

Figure 2 CT scan demonstrating anterior mediastinal mass.

Please cite this article in press as: Jennings S, et al. Successful Giant Thymic Cyst Removal: Case report and review of the literature. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.013

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Figure 3 Thin-walled unilocular cyst with fatty thymic tissue.

Figure 4 Cuboidal corpuscles.

epithelium containing Hassall

an approximately 1.8 kg benign thymic cyst (congenital origin). The cyst was lined by bland cuboidal epithelium of variable thickness composed of fibrous connective and adipose tissue. Aggregates of lymphoid tissue including Hassall corpuscles confirmed thymic origin (Figure 4). No malignancy was identified. Rapid atrial fibrillation developed, requiring chemical cardioversion, and complicating convalescence. On six-week review the patient was completely asymptomatic. Repeat lung function testing showed 30% improvement in FEV1/FVC ratio. The patient will continue to be monitored in general practice. Repeat CXR can be seen in Figure 1b.

cysts reported is important. Besides local compressive effects, a report of a large thymic cyst that was involved in a watch and wait approach resulted in spontaneous mediastinal infection. The patient required emergency thoracotomy for removal of the cyst, mediastinal abscess and pleural effusion [2]. In retrospect, the authors conceded a conservative approach unsuitable and early cardiothoracic surgical referral would have avoided significant morbidity. Conservative treatments for large benign thymic cysts have been reported as being of low-yield. The majority of these cysts are encased by dense fibro-fatty tissue or a thick capsule resulting in diagnostic uncertainty with fluid being acellular in nature [3,8]. Percutaneous drainage may be appropriate for mildly symptomatic cysts or individuals not suitable for surgery. It was felt by the surgical team that given the natural history of cysts, the risk of recurrence was significant. It was also felt this would be inappropriate, subjecting the patient to repeated painful drainage procedures. The patient also indicated a need for conclusive resolution. There is currently no report in the literature of definitive, successful and conclusive treatment being achieved by percutaneous methods. Surgical excision, via median sternotomy, thoracotomy or video-assisted techniques is necessary for definitive diagnosis, treatment and elimination of recurrence. Given the size of the mass and the lack of initial certain diagnosis, it was felt that en-bloc removal of the cyst was preferable to avoid potential complications, such as perforation, content spillage or incomplete excision. As demonstrated in this case, giant thymic cysts also have a significant impact on respiratory physiology. Removal of giant cysts allows for re-expansion of chronically collapsed lung and improvement in both respiratory ventilation and perfusion. No local recurrence of an excised thymic cyst has been reported to date [1–3]. A watch and wait approach may be employed, but it is stressed that definitive diagnosis is only achieved by excision [8]. Definitive diagnosis is important as bronchogenic and acquired multi-locular cysts resulting from inflammation and neoplasm require exclusion [1,3,6]. It must also be noted that thymic cysts can coexist with varied malignancies such as teratoma, Hodgkin disease, thymoma, thymic carcinoma or neurofibromatosis [6]. The cystic variant of thymoma may also be present. Although extremely rare, 2 cases of thymoma with intracystic dissemination have been recorded arising from a unilocular cyst. Up to 40% of thymomas can also undergo cystic change [9]. Haemorrhage into large cysts is also a major concern. Although reported in younger individuals, thymic cysts large enough to cause compressive symptoms are usually seen in those aged 40-60 yrs [1]. Age and declining physiological reserve are paramount when deciding treatment options.

Discussion A consensus and evidence-based guidelines on the management of giant benign thymic cysts is currently lacking. The literature consists of case reports with no large series available. However, the potential sequelae of benign giant thymic

Conclusion In conclusion, despite lack of consensus of management and lack of information in regarding benign thymic cysts,

Please cite this article in press as: Jennings S, et al. Successful Giant Thymic Cyst Removal: Case report and review of the literature. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.013

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the determining factors we put forward are tumour size, symptomatology and patient factors. Large benign thymic cysts exerting compressive effects we feel should be removed, guaranteeing definitive diagnosis and prevent recurrence. Early referral to a cardiothoracic surgeon with a plan to excise should be sought. Removal also confirms tumour benignity, eliminates painful, repeated draining procedures and the risk of spontaneous bacterial infection and mediastinitis. By sharing this report it is hoped fellow clinicians can formulate the most effective treatment strategy when dealing with this uncommon and potentially serious condition. There is also a need for further reporting of this condition to establish an evidence-based approach for future care.

Disclosures Nil.

Conflict of Interest

References [1] Bouma W, Klinkenberg T, Van De Wauwer C, Timens W, Mariani M. Removal of a giant intrathoracic cyst from the anterior mediastinum. Journal of Cardiothoracic Surgery 2014;9(152). [2] Nanguzgambo A, Pike M, Page R, Benfield G, McKeon D. Spontaneous infection of a stable mediastinal cystic mass: A case report. Cases Journal 2008;1(126). [3] Butcovan D. Medistinal Thymic Cyst case report and review of the literautre [30 Oct 2014]. Available from: http://www.umfiasi.ro/ Rezidenti/suporturidecurs/Facultatea de Medicina/ANATOMIE PATOLOGICA/2 Mediastinal thymic cyst.pdf. [4] Dyer N. Cystic thymomas and thymic cysts. Thorax 1967;22:408–21. [5] KouerinisI, Zografos G, Exarchos D, Silimingas N, Argiriou M, Manoussaridis J, et al. A huge posteromedial mediastinal cyst complicated with vertebral dislodgment. World Journal of Surgical Oncology 2006;4(56.). [6] Constantacos C, Lawson N, Votanopoulos K, Olutoye O, Eldin K, Feigin R. Giant thymic cyst in left lower hemithorax of a healthy teenaged athlete. The American Association for Thoracic Surgery 2007;134 (5):1373–4. [7] Rastegar H, Arger P, Harken A. Evaluation and therapy of mediastinal thymic cyst. Am Surg 1980;46(4):236–8. [8] Myung R, Leshnower B, Miller J. Mediastinal Coelomic Cyst. The Annals of Thoracic Surgery 2010;91(6):1967–9. [9] Kitami A, Kamio Y, Uematsu S, Sato Y, Kadokura M. Thymoma with intracystic dissemination arising in a unilocular thymic cyst. General Thoracic and Cardiovascular Surgery 2007;55(7):281–3.

None to declare

Please cite this article in press as: Jennings S, et al. Successful Giant Thymic Cyst Removal: Case report and review of the literature. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.013

Successful Giant Thymic Cyst Removal: Case Report and Review of the Literature.

Giant thymic cysts are a rare clinical entity evolving from smaller benign thymic cysts over many years. Benign thymic cysts account for approximately...
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