Case Report

1. Retrosternal goiter and thymic carcinoid: A rare co-existence
Abdulsalam Yaseen Taha a, *
, Nezar A. Almahfooz b
, Hassanain H. Khudair c
a
Department of Thoracic and Cardiovascular Surgery, Sulaimaniyah Teaching Hospital and School of Medicine, Faculty of Medical
Sciences, University of Sulaimaniyah, Sulaimaniyah, Iraq
b
Department of General Surgery, Farouk Medical City, Sulaimaniyah, Iraq
c
Department of Pathology, School of Medicine, University of Sulaimaniyah, Sulaimaniyah, Iraq
a r t i c l e i n f o
Article history:
Received 19 September 2017
Received in revised form 14 October 2017
Accepted 10 November 2017
Available online xxx
Keywords:
Retrosternal goiter
Carcinoid tumor
Thymus
Argentaffin (Kultschitzky) cells
Anterior mediastinum
a b s t r a c t
Retrosternal goiter is diagnosed when more than 50% of the thyroid gland extends below
the thoracic inlet. Surgery is the treatment of choice. Carcinoid tumor of thymus gland is
very rare. Although both conditions develop in the anterior mediastinum, literature search
revealed no patient having both lesions at the same time. Reported herein, is a 55-year old
Iraqi man with retrosternal multinodular goiter and a localized solitary primary thymic
carcinoid. Thymic tumor was simultaneously removed along right thyroid lobectomy via
median sternotomy extended to the neck. Early outcome was good. The patient had no
evidence of recurrence after surgery.
© 2017 The Egyptian Society of Cardio-thoracic Surgery. Publishing services by Elsevier B.V.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/
licenses/by-nc-nd/4.0/).
1. Introduction
Retrosternal goiter (RSG) was first described by Haller in 1749 [1]. Surgery is the treatment of choice for RSG due to
possible existence of thyroid malignancy and the potential for airway compromise [2]. Removal can mostly be achieved
through a transcervical approach. However, median sternotomy may sometimes be necessary [1,2]. Beside lymphocytes,
thymocytes and epithelial stroma, thymus gland also contains argentaffin (Kulchitsky) cells which give origin to carcinoid
tumors [3e6]. The term carcinoid was introduced by Oberndorfer (1907) to designate a tumor which histologically resembled
an undifferentiated carcinoma but behaved in a benign fashion [4]. Rosai and Higa identified thymic carcinoid (TC) as a
separate entity from thymoma in 1972 [6,7]. Although both TC tumor and the vast majority of RSG are located in the anterior
mediastinum [2], co-existence of both lesions was not found on literature review.
2. Case presentation
A man of 55 admitted to Sulaimaniyah Teaching Hospital (STH), Sulaimaniyah, Iraq on 9th October 2012 with an anterior
neck mass of 30 years duration associated with effort dyspnea and chest pain in the proceeding few months but no sweating,
flushing, or diarrhea. Physical examination revealed a visible thyroid enlargement mainly of the right lobe with increasing
* Corresponding author.
E-mail addresses: salamyt_1963@hotmail.com, abdulsalam.taha@univsul.edu.iq (A.Y. Taha), almahfoozna@gmail.com (N.A. Almahfooz), hhk1970@gmail.
com (H.H. Khudair).
Peer review under responsibility of The Egyptian Society of Cardio-thoracic Surgery.
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https://doi.org/10.1016/j.jescts.2017.11.004
1110-578X/© 2017 The Egyptian Society of Cardio-thoracic Surgery. Publishing services by Elsevier B.V. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Journal of the Egyptian Society of Cardio-Thoracic Surgery xxx (2017) 1e4
Please cite this article in press as: Taha AY, et al., Retrosternal goiter and thymic carcinoid: A rare co-existence, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2017), https://doi.org/10.1016/j.jescts.2017.11.004

2. dyspnea on arm elevation. Chest radiograph showed left-sided tracheal deviation with a rounded left hilar mass (Fig. 1-A).
Electrocardiography, echocardiography, fiberoptic bronchoscopy and thyroid function tests were normal [T3 ¼ 1.6 nmol/L
(normal value: 0.9e2.8), T4 ¼ 90 nmol/L (normal value: 58e161) and TSH ¼ 1.2 mIU/L (normal value: 0.5e4.7)]. Chest CT scan
(Fig. 1-B) showed a large well-defined solid anterior mediastinal mass while neck CT scan (Fig. 1-C) revealed a large complex
thyroid mass with calcification displacing the trachea to the left side. Fine needle aspiration cytology (FNAC) revealed benign
follicular epithelial thyroid cells. The patient underwent one-session surgery. Endotracheal intubation wasn't difficult. Once
the sternum was divided; the mediastinal mass was found loosely adherent to the great vessels and easily resected (Fig. 2-A).
The incision was then extended into the neck. Thyroid enlargement was huge extending down into the mediastinum. Right
thyroid lobectomy was performed (Fig. 2-B). After securing hemostasis and placement of drains, the wound was closed.
Postoperatively, the trachea regained its central position (Fig. 2-C). Histopathological exam revealed a colloid goiter and TC
tumor (Fig. 3).
3. Discussion
In this report, a man of 55 with TC tumor and RSG is described. Preoperative work up failed to arrive at definite tissue
diagnoses. Although most RSGs can be removed via a cervical incision, median sternotomy was chosen due to co-existence of
the anterior mediastinal mass. Worthy to note, both thymus and thyroid glands normally contain argentaffin cells. They are
named C-cells in the thyroid gland and can give rise to thyroid medullary carcinoma (TMC). However, the chief locations of
argentaffin cells are the gastrointestinal and respiratory tracts. In this case, neither TMC nor gastro-intestinal or bronchial
Fig. 1. A: Preoperative CXR -B: Chest CT scan -C: Cervical CT scan.
A.Y. Taha et al. / Journal of the Egyptian Society of Cardio-Thoracic Surgery xxx (2017) 1e42
Please cite this article in press as: Taha AY, et al., Retrosternal goiter and thymic carcinoid: A rare co-existence, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2017), https://doi.org/10.1016/j.jescts.2017.11.004

3. carcinoid tumors were present; hence, the tumor was solitary and primary. Moreover, the tumor did not invade nearby
structures or metastasized distantly. The carcinoid syndrome that usually results from serotonin secretion was absent.
RSG is said to exist in up to one fifth of patients undergoing thyroidectomy, 90% of which are located in the anterior
mediastinum. Diagnosis of RSG is most frequently made in the 5th or 6th decades with symptoms related to tracheal or
esophageal compression by the slow-growing thyroid gland [2]. Similarly, this 55-year old patient had a long-standing goiter
associated with shortness of breath.
Primary TC tumor is very rare. Just 100 cases have been reported world-wide by 1994 [6]. From 1995 to 2010, Ahn S et al.
from South Korea, found only 18 cases; most of them were males (male/female ratio, 15/6) [7]. The patients may be
Fig. 2. A: Thymic Carcinoid -B: Right thyroid lobectomy -C: Post-operative Chest Film.
Fig. 3. Microscopic Appearance of TC Tumor; nests of uniform small round cells, with small round nuclei and pink cytoplasm, with no pleomorphism and no
necrosis, with rosette-like formation.
A.Y. Taha et al. / Journal of the Egyptian Society of Cardio-Thoracic Surgery xxx (2017) 1e4 3
Please cite this article in press as: Taha AY, et al., Retrosternal goiter and thymic carcinoid: A rare co-existence, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2017), https://doi.org/10.1016/j.jescts.2017.11.004

4. asymptomatic or have compression symptoms such as chest pain, dyspnea, cough and superior vena cava obstruction.
Cushing's syndrome may be seen in half the patients [7]. Large carcinoid tumor may cause flushing, cyanosis, and hyper-
tension ‘carcinoid syndrome’ due to secretion of serotonin [4]. Many TCs are small (<2 cm) and compose of epithelial cells
with small uniform nuclei and acidophilic or vacuolated cytoplasm [3]. They grow slowly and frequently invade the lung and
pericardium [5]. FNAC may be diagnostic. However, these tumors are usually diagnosed by surgical exploration or media-
stinoscopy [6]. Complete resection of the tumor remains the most important prognostic factor [8]. Despite extensive
resection, recurrence may occur years later [6].
TMC has morphological and behavioral characteristics similar to carcinoid [5]; Clague JE et al. had reported the case of a 21
year old man in whom multifocal bronchial carcinoid was diagnosed initially, but at necropsy metastatic TMC was found [9].
Ectopic thyroid tissue may be found in the thymus; Kesici et al. described a 3 Â 4 cm ectopic thyroid tissue in the thymus gland
in a 49-year old lady after total thyroidectomy [10]. Noteworthy, the presented patient has neither TMC nor ectopic thyroid.
4. Conclusions
Co-existence of RSG and the rare thymic carcinoid tumor is very rare. Surgery is thought to be curative in the present case.
Author contributions
1. Abdulsalam Y Taha: received and surgically managed the case together with Dr. Nezar A Almahfooz, reviewed the liter-
ature and wrote the manuscript.
2. Nezar A Almahfooz: joined Prof. Abdulsalam Y Taha in doing surgery, approved the manuscript.
3. Hassanain H Khudair did the histopathological exam and approved the manuscript.
References
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A.Y. Taha et al. / Journal of the Egyptian Society of Cardio-Thoracic Surgery xxx (2017) 1e44
Please cite this article in press as: Taha AY, et al., Retrosternal goiter and thymic carcinoid: A rare co-existence, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2017), https://doi.org/10.1016/j.jescts.2017.11.004