Fetal and Pediatric Pathology
ISSN: 1551-3815 (Print) 1551-3823 (Online) Journal homepage: http://www.tandfonline.com/loi/ipdp20
Cervical Thymic Cyst in Childhood: A Case Report Marta Betti, Nasim Hanie Hoseini, Alessandra Martin, Annamaria Buccoliero, Antonio Messineo & Marco Ghionzoli To cite this article: Marta Betti, Nasim Hanie Hoseini, Alessandra Martin, Annamaria Buccoliero, Antonio Messineo & Marco Ghionzoli (2015) Cervical Thymic Cyst in Childhood: A Case Report, Fetal and Pediatric Pathology, 34:1, 65-69, DOI: 10.3109/15513815.2014.966181 To link to this article: http://dx.doi.org/10.3109/15513815.2014.966181
Published online: 20 Oct 2014.
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Date: 04 December 2016, At: 01:08
Fetal and Pediatric Pathology, 34:65–69, 2015 C Informa Healthcare USA, Inc. Copyright ISSN: 1551-3815 print / 1551-3823 online DOI: 10.3109/15513815.2014.966181
ORIGINAL ARTICLE
Cervical Thymic Cyst in Childhood: A Case Report Marta Betti,1 Nasim Hanie Hoseini,1 Alessandra Martin,1 Annamaria Buccoliero,2 Antonio Messineo,1 and Marco Ghionzoli1 1
University of Florence and Children’s University Hospital, Department of emergency, Critical Area and Pediatric surgery, Viale Pieraccini 24, Florence, Italy; 2 University of Florence, Department of Clinical and Experimental Medicine, Florence, Italy
Cervical thymic cysts are rare cervical masses which usually present in the first decade of life accounting for 0.3% - 1% of all congenital neck masses. Due to its rarity thymic cysts are seldom included in the differential diagnosis of cystic cervical mass and are often mistaken for more common conditions such as branchial cleft cyst or cystic hygroma. Herein we present a case of a 6-year-old girl with a right lateral cervical cystic mass with multiple septae extended from mandibular angle along the neurovascular bundle of the neck which revealed to be a thymic cyst at histopathology. Keywords: cervical mass, thymic cyst, asymptomatic
INTRODUCTION Cervical thymic cysts (CTC) are a rare cause of cervical mass: various reports indicate an incidence varying from 0.3% to 1% of all congenital neck masses [1, 2] and they usually present in the first decade of life being more common in males [2]. They usually manifest as a slowly growing asymptomatic mass in the region between the gonial angle of the mandible and the sternum, in proximity of the carotid sheath. The main differential diagnoses for cystic masses in the neck are thyroglossal duct cysts, branchial cleft cysts, cystic hygromas, epidermoid cysts and enlarges lymph nodes [2]. Current literature agrees upon the fact that CTC, although rare, should be included as a differential diagnosis in pediatric patients presenting with a cervical neck mass. In this report we present a case of an asymptomatic CTC in a child and we discuss its clinical relevance and management. CASE REPORT A 6-year-old asymptomatic girl was followed up at our department for a lateral neck mass that was incidentally diagnosed two years before on a neck ultrasound (US) scan performed because of a contralateral cervical lymphadenopathy. Clinical Received 2 July 2014; Revised 4 September 2014; accepted 10 September 2014. Address correspondence to Dr Marco Ghionzoli MD PhD, University of Florence and Children’s University Hospital, Department of Pediatric Surgery, viale Pieraccini 24, Florence, Italy. E-mail: [email protected]
M. Betti et al.
Figure 1. a–d MRI-imaging. a: STIR anterior-posterior coronal sequence b: STIR coronal sequence showing the thymic cyst c: T1-weighted axial picture which demonstrates a thymic cyst on the right side of the neck d: STIR sagittal sequence.
examination showed a barely palpable lateral cervical mass with no signs of swelling, nor altered skin. Perioperative blood tests were normal. MRI scan demonstrated a right lateral-cervical cystic mass under the sternocleidomastoid muscle characterized by multiple septae, measuring 69 × 25 × 19 mm, extended from mandibular angle down to the sternum along the ipsilateral neurovascular bundle of the neck. T1 and T2 weighted images showed inhomogeneous signal without a significant enhancement after contrast injection compatible with a high protein fluid content within the cyst (Figure 1a–d). Surgical excision was performed under general anesthesia. A longitudinal skin incision along the anterior edge of sternocleidomastoid muscle was performed. A soft cystic mass, lying next to the neurovascular bundle which was extended from the mastoid region down to superior mediastinum was bluntly dissected and removed (Figure 2a–d). A drain was left in place in the surgical bed which was removed 24 hours after surgery and the patient was discharged after two days. On macroscopic examination the surgical specimen consisted of a solid-cystic mass which appeared multilocular with a dense brown fluid content. Histopathological analysis revealed that the solid part of the lesion consisted of thymic tissue which Fetal and Pediatric Pathology
Cervical Thymic Cyst in Childhood: A Case Report
Figure 2. Images from the surgical excision. a–b: The thymic cyst is bluntly dissected from the neurovascular bundle of the neck. c: The thymic cyst specimen d: The surgical bed after dissection. Carotid artery, vagus nerve and internal jugular vein are shown.
included Hassall’s corpuscles. In proximity of this solid fragment, a cyst defined by a fibrous wall lined with cuboidal epithelium was found. In addition, the cystic wall contained a lymphocyte infiltrate and several cholesterol granulomas; cholesterol crystals were also present in the luminal fluid (Figure 3 – black asterisks). The excision was complete and patient did not show any recurrence at postoperative follow up which consisted of a clinical evaluation and neck US. DISCUSSION The thymus develops during the 6th gestational week from the ventrolateral surface of the third pharyngeal pouch. Solid buds originate from the 7th week of gestation when proliferation of endodermal cells begins to descend toward the mediastinum. The primordial tract usually involves although remnants can appear anywhere along the tract of descent giving rise to thymic cysts, which usually stand between pyriform recesses and mediastinum [3]. CTC usually presents with variable size during the first decade of life, debuting as a slow growing painless mass in the laterocervical area which is extended up to mediastinum in about half of cases [2]. About 90% of the cases are asymptomatic [4], although a small amount of larger CTC is able to compress the neighboring structures causing symptoms such as dysphagia, dyspnea and hoarseness of voice [2]. CTC occur mostly on the left side (up to 70% of the cases) and there is a slight predominance among males [4]; interestingly enough we report a female patient with CTC on the right side of the neck. Imaging, surgical findings and histopathological correlation play an important role in diagnosing a thymic cyst [2]. C Informa Healthcare USA, Inc. Copyright
M. Betti et al.
Figure 3. Thymic tissue (black arrows) with the contiguous cyst; in the lumen of the cyst there were cholesterol crystals. The cystic wall (black arrowheads) was composed of fibrous tissue lined with a bland cuboidal epithelium and contained a lymphocytic infiltrate and cholesterol granulomas (black asterisks).
Cervical ultrasound scan with doppler as first level imaging is able to focus cyst characteristics such as presence of septae, ipo-anechoic content, its relationship with the surrounding structures and hints of vascularization which are better defined by second level imaging [5]. Although the use of CT scan has been reported [5] contrast MRI is the imaging of choice due to absence of biological hazard and enhanced soft tissue imaging [3, 6]. Surgery has both a diagnostic and therapeutic role and a complete excision is advised. No immunological deficiency after removal of a CTC is reported; this has been explained by the fact that the CTC does not contain any functional thymic tissue [4]. Although unusual in childhood, complications which should be taken into consideration in the long term are thymic carcinoma and myasthenia gravis [4, 6]. Concerning histopathology two varieties of CTC have been described: thymopharyngeal duct cyst and cysts arising from degeneration of Hassall’s corpuscles within ectopic thymic remnants [2]. At macroscopic overview, CTC are usually soft, uni or multilocular masses which contain a brown fluid and sometimes semisolid necrotic debris and cholesterol crystals [4]. The cyst wall is lined with an epithelium that can be either squamous, cuboidal or columnar and thymic elements can be found in contact with the cyst wall; Hassall’s corpuscles are also evident. These features are essential in establishing the histopathological diagnosis [4]. In conclusion, adequate reporting of cases is necessary to improve the CTC awareness. A diagnosis should be considered for children presenting with an asymptomatic cervical neck mass. Fetal and Pediatric Pathology
Cervical Thymic Cyst in Childhood: A Case Report
Declaration of Interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. REFERENCES [1] Niranjan J, Santosh K, Prabhakar G. Multilocated cervical thymic cyst. J Indian Assoc Pedatr Surg 2011;16(1):24–25. [2] Shenoy V, Kamath MP, Hegde MC, et al. Case report cervical thymic cyst: A rare differential diagnosis in lateral neck swelling. Otolaryngology 2013: 350502 [3] Kuperan AB, Quraishi H a, Shah AJ, et al. Thymopharyngeal duct cyst: A case presentation and literature review. The Laryngoscope 2010 Jan;120 Suppl:S226. [4] Ozturk ¨ H, Karnak I, Deveci S, et al. Multilocular cervical thymic cyst: an unusual neck mass in children. Int J Pediatr Otorhinolaryngol 2001 Dec 1;61(3):249–252. [5] Ballal HS, Mahale A, Hegde V, Shetty R, Bhavikatti M. Cervical thymic cyst. Indian J Radiol Imaging 1999;9:187–189. [6] Tandon A, Tandon R, Chandrashekhar M, et al. Cervical ectopic thymic cyst: A rare preoperative diagnosis. BMJ Case Reports 2011 Oct; doi: 10.1136/bcr.05.2011.4250.
C Informa Healthcare USA, Inc. Copyright
ISSN: 1551-3815 (Print) 1551-3823 (Online) Journal homepage: http://www.tandfonline.com/loi/ipdp20
Cervical Thymic Cyst in Childhood: A Case Report Marta Betti, Nasim Hanie Hoseini, Alessandra Martin, Annamaria Buccoliero, Antonio Messineo & Marco Ghionzoli To cite this article: Marta Betti, Nasim Hanie Hoseini, Alessandra Martin, Annamaria Buccoliero, Antonio Messineo & Marco Ghionzoli (2015) Cervical Thymic Cyst in Childhood: A Case Report, Fetal and Pediatric Pathology, 34:1, 65-69, DOI: 10.3109/15513815.2014.966181 To link to this article: http://dx.doi.org/10.3109/15513815.2014.966181
Published online: 20 Oct 2014.
Submit your article to this journal
Article views: 81
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipdp20 Download by: [FU Berlin]
Date: 04 December 2016, At: 01:08
Fetal and Pediatric Pathology, 34:65–69, 2015 C Informa Healthcare USA, Inc. Copyright ISSN: 1551-3815 print / 1551-3823 online DOI: 10.3109/15513815.2014.966181
ORIGINAL ARTICLE
Cervical Thymic Cyst in Childhood: A Case Report Marta Betti,1 Nasim Hanie Hoseini,1 Alessandra Martin,1 Annamaria Buccoliero,2 Antonio Messineo,1 and Marco Ghionzoli1 1
University of Florence and Children’s University Hospital, Department of emergency, Critical Area and Pediatric surgery, Viale Pieraccini 24, Florence, Italy; 2 University of Florence, Department of Clinical and Experimental Medicine, Florence, Italy
Cervical thymic cysts are rare cervical masses which usually present in the first decade of life accounting for 0.3% - 1% of all congenital neck masses. Due to its rarity thymic cysts are seldom included in the differential diagnosis of cystic cervical mass and are often mistaken for more common conditions such as branchial cleft cyst or cystic hygroma. Herein we present a case of a 6-year-old girl with a right lateral cervical cystic mass with multiple septae extended from mandibular angle along the neurovascular bundle of the neck which revealed to be a thymic cyst at histopathology. Keywords: cervical mass, thymic cyst, asymptomatic
INTRODUCTION Cervical thymic cysts (CTC) are a rare cause of cervical mass: various reports indicate an incidence varying from 0.3% to 1% of all congenital neck masses [1, 2] and they usually present in the first decade of life being more common in males [2]. They usually manifest as a slowly growing asymptomatic mass in the region between the gonial angle of the mandible and the sternum, in proximity of the carotid sheath. The main differential diagnoses for cystic masses in the neck are thyroglossal duct cysts, branchial cleft cysts, cystic hygromas, epidermoid cysts and enlarges lymph nodes [2]. Current literature agrees upon the fact that CTC, although rare, should be included as a differential diagnosis in pediatric patients presenting with a cervical neck mass. In this report we present a case of an asymptomatic CTC in a child and we discuss its clinical relevance and management. CASE REPORT A 6-year-old asymptomatic girl was followed up at our department for a lateral neck mass that was incidentally diagnosed two years before on a neck ultrasound (US) scan performed because of a contralateral cervical lymphadenopathy. Clinical Received 2 July 2014; Revised 4 September 2014; accepted 10 September 2014. Address correspondence to Dr Marco Ghionzoli MD PhD, University of Florence and Children’s University Hospital, Department of Pediatric Surgery, viale Pieraccini 24, Florence, Italy. E-mail: [email protected]
M. Betti et al.
Figure 1. a–d MRI-imaging. a: STIR anterior-posterior coronal sequence b: STIR coronal sequence showing the thymic cyst c: T1-weighted axial picture which demonstrates a thymic cyst on the right side of the neck d: STIR sagittal sequence.
examination showed a barely palpable lateral cervical mass with no signs of swelling, nor altered skin. Perioperative blood tests were normal. MRI scan demonstrated a right lateral-cervical cystic mass under the sternocleidomastoid muscle characterized by multiple septae, measuring 69 × 25 × 19 mm, extended from mandibular angle down to the sternum along the ipsilateral neurovascular bundle of the neck. T1 and T2 weighted images showed inhomogeneous signal without a significant enhancement after contrast injection compatible with a high protein fluid content within the cyst (Figure 1a–d). Surgical excision was performed under general anesthesia. A longitudinal skin incision along the anterior edge of sternocleidomastoid muscle was performed. A soft cystic mass, lying next to the neurovascular bundle which was extended from the mastoid region down to superior mediastinum was bluntly dissected and removed (Figure 2a–d). A drain was left in place in the surgical bed which was removed 24 hours after surgery and the patient was discharged after two days. On macroscopic examination the surgical specimen consisted of a solid-cystic mass which appeared multilocular with a dense brown fluid content. Histopathological analysis revealed that the solid part of the lesion consisted of thymic tissue which Fetal and Pediatric Pathology
Cervical Thymic Cyst in Childhood: A Case Report
Figure 2. Images from the surgical excision. a–b: The thymic cyst is bluntly dissected from the neurovascular bundle of the neck. c: The thymic cyst specimen d: The surgical bed after dissection. Carotid artery, vagus nerve and internal jugular vein are shown.
included Hassall’s corpuscles. In proximity of this solid fragment, a cyst defined by a fibrous wall lined with cuboidal epithelium was found. In addition, the cystic wall contained a lymphocyte infiltrate and several cholesterol granulomas; cholesterol crystals were also present in the luminal fluid (Figure 3 – black asterisks). The excision was complete and patient did not show any recurrence at postoperative follow up which consisted of a clinical evaluation and neck US. DISCUSSION The thymus develops during the 6th gestational week from the ventrolateral surface of the third pharyngeal pouch. Solid buds originate from the 7th week of gestation when proliferation of endodermal cells begins to descend toward the mediastinum. The primordial tract usually involves although remnants can appear anywhere along the tract of descent giving rise to thymic cysts, which usually stand between pyriform recesses and mediastinum [3]. CTC usually presents with variable size during the first decade of life, debuting as a slow growing painless mass in the laterocervical area which is extended up to mediastinum in about half of cases [2]. About 90% of the cases are asymptomatic [4], although a small amount of larger CTC is able to compress the neighboring structures causing symptoms such as dysphagia, dyspnea and hoarseness of voice [2]. CTC occur mostly on the left side (up to 70% of the cases) and there is a slight predominance among males [4]; interestingly enough we report a female patient with CTC on the right side of the neck. Imaging, surgical findings and histopathological correlation play an important role in diagnosing a thymic cyst [2]. C Informa Healthcare USA, Inc. Copyright
M. Betti et al.
Figure 3. Thymic tissue (black arrows) with the contiguous cyst; in the lumen of the cyst there were cholesterol crystals. The cystic wall (black arrowheads) was composed of fibrous tissue lined with a bland cuboidal epithelium and contained a lymphocytic infiltrate and cholesterol granulomas (black asterisks).
Cervical ultrasound scan with doppler as first level imaging is able to focus cyst characteristics such as presence of septae, ipo-anechoic content, its relationship with the surrounding structures and hints of vascularization which are better defined by second level imaging [5]. Although the use of CT scan has been reported [5] contrast MRI is the imaging of choice due to absence of biological hazard and enhanced soft tissue imaging [3, 6]. Surgery has both a diagnostic and therapeutic role and a complete excision is advised. No immunological deficiency after removal of a CTC is reported; this has been explained by the fact that the CTC does not contain any functional thymic tissue [4]. Although unusual in childhood, complications which should be taken into consideration in the long term are thymic carcinoma and myasthenia gravis [4, 6]. Concerning histopathology two varieties of CTC have been described: thymopharyngeal duct cyst and cysts arising from degeneration of Hassall’s corpuscles within ectopic thymic remnants [2]. At macroscopic overview, CTC are usually soft, uni or multilocular masses which contain a brown fluid and sometimes semisolid necrotic debris and cholesterol crystals [4]. The cyst wall is lined with an epithelium that can be either squamous, cuboidal or columnar and thymic elements can be found in contact with the cyst wall; Hassall’s corpuscles are also evident. These features are essential in establishing the histopathological diagnosis [4]. In conclusion, adequate reporting of cases is necessary to improve the CTC awareness. A diagnosis should be considered for children presenting with an asymptomatic cervical neck mass. Fetal and Pediatric Pathology
Cervical Thymic Cyst in Childhood: A Case Report
Declaration of Interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. REFERENCES [1] Niranjan J, Santosh K, Prabhakar G. Multilocated cervical thymic cyst. J Indian Assoc Pedatr Surg 2011;16(1):24–25. [2] Shenoy V, Kamath MP, Hegde MC, et al. Case report cervical thymic cyst: A rare differential diagnosis in lateral neck swelling. Otolaryngology 2013: 350502 [3] Kuperan AB, Quraishi H a, Shah AJ, et al. Thymopharyngeal duct cyst: A case presentation and literature review. The Laryngoscope 2010 Jan;120 Suppl:S226. [4] Ozturk ¨ H, Karnak I, Deveci S, et al. Multilocular cervical thymic cyst: an unusual neck mass in children. Int J Pediatr Otorhinolaryngol 2001 Dec 1;61(3):249–252. [5] Ballal HS, Mahale A, Hegde V, Shetty R, Bhavikatti M. Cervical thymic cyst. Indian J Radiol Imaging 1999;9:187–189. [6] Tandon A, Tandon R, Chandrashekhar M, et al. Cervical ectopic thymic cyst: A rare preoperative diagnosis. BMJ Case Reports 2011 Oct; doi: 10.1136/bcr.05.2011.4250.
C Informa Healthcare USA, Inc. Copyright
