A rare case of supraclavicular region: Ectopic

thymoma
Abstract

Background: Ectopic thymoma is a rare tumor that can be seen in the cervical region and
caused by ectopic thymic tissue formed due to disruption in the migration of the thymus
during the fetal period. It is most commonly seen in middle-aged men. Although rare, it is
important to be aware that thymoma may also be present in the differential diagnosis of
cervical masses.

Case presentation: A 53-year-old male patient applied to our clinic due to swelling on the
left side of the neck that had been present for 1 year. A mass approximately 6 cm in size was
detected in the left supraclavicular region. Neck MRI also revealed an oval heterogeneous
mass containing mixed components of soft tissue and fat density. Fine needle aspiration was
performed and a diagnosis of ectopic hamartomatous thymoma was made. The patient then
underwent major total surgical resection of the tumor under field block anesthesia, without
any large residual tumor. And postoperative pathological diagnosis of type A thymoma was
made.

Conclusion: Although ectopic thymomas in the cervical region are rare, thymomas may be
considered as a differential diagnosis for a supraclavicular masses.

Keywords: Ectopic thymoma, Ectopic thymus , soft tissue tumor, head and neck

Introduction
The thymus gland is a structure located in the anterior mediastinum and responsible for
cellular immunity. Although thymic tumors are rare, they are responsible for approximately
47% of anterior mediastinal tumors. Additionally, it may be found ectopically outside the
anterior mediastinum, including the neck, middle or posterior mediastinum, lung, and
pleura. Ectopic thymomas are thought to arise from dispersed thymic tissue that cannot
migrate into the anterior-superior mediastinum. (1) According to Shields' classification,
thymic tumors are divided into four main groups. These; epithelial cell tumors (thymomas
and thymic carcinomas), tumors of neuroendocrine origin, thymolipomas and other rare
tumors. (neuroblastoma, ganglioneuroblastoma, malignant melanoma, thymic hemangioma
and myoid tumors). Although they are mostly well-circumscribed, encapsulated and slow-
growing tumors, it has been reported that they may coexist with myasthenia gravis and
cause systemic diseases such as erythroid hypoplasia and hypogammaglobulinemia (2). In
this study, we describe the clinicopathological features of an ectopic timoma located in the
left supraclavicular region and review the literatüre on this subject.

Case presentation

A 53-year-old male patient presented with a 1-year history of a slow-growing painless mass
located in the left supraclavicular region with mild tenderness on palpation. He was
diagnosed with FMF and type 1 diabetes. He was using oral antidiabetics and colchicine. He
had a history of appendectomy 20 years ago and an operation for ingunal hernia 1 year ago.
His older sister was diagnosed with leukemia. He did not use alcohol or tobacco. Routine
laboratory data were all within normal ranges. Physical examination revealed a 6.0 cm oval,
mobile mass in the left supraclavicular region. The hard mass was slightly tender and had
clear boundaries on palpation. Neck MRI also revealed an oval heterogeneous mass
containing mixed components of soft tissue and fat density. The image was enhanced with
the application of contrast. There was no abnormality on the chest X-ray. Fine needle
aspiration was performed and a condition with undetermined cytopathology was reported.
A second fine needle aspiration was performed and a diagnosis of ectopic hamartomatous
thymoma was made. The patient then underwent major total surgical resection of the tumor
under field block anesthesia, without any large residual tumor. During the surgery, a well-
circumscribed oval tumor measuring 7.0 cm x 5 cm x 2.5 cm, without invasion, was detected
under the SCM muscle in the left supraclavicular region. The tumor caused moderate dorsal
displacement of the left carotid artery due to its pressure. Additionally, there was no
evidence of tumor invasion into adjacent tissues. The tumor was not connected to the
thyroid, clavicle, or mediastinum. The patient's postoperative period was uneventful. The
pathological tissue was in the form of a nodular lesion, 7 x 5 x 3.5 cm in size, yellow brown in
color, with a shiny and smooth surface. In cross-sections, large areas have a yellow color and
a regular appearance, and there are cream-colored, fibrotic-looking areas scattered among
them and occasionally bleeding areas.

Immunohistochemical staining showed nuclear positivity for p63 and CK7 , CK19 due to
diffuse and intense cytoplasmic reactivity of both spindle cell and epithelial components.
Spindle cells are also negative for spindle muscle actin, desmin, S-100 protein; CK5/6 , p63
was positive . These general clinicopathological features found in the patient met the
diagnostic criteria for ectopic thymoma. The final histopathological diagnosis was Type A
ectopic thymoma; no microinvasion was detected and surgical margins were negative. It
was thought that the tumor developed on the basis of lipofibroadenoma.

Şekil 1/ Cervical mass detected in the left side of supraclavicular region by Magnetic Resonance Imaging (MRI)

Şekil 2/ Enhanced neck MRI axial section showed a solitary, well-defined nodule in the left supraclavicular region.
Şekil 3

Şekil 4

Discussion

Cervical thymoma is caused by ectopic thymic tissue formed due to disruption in the
migration of the thymus during the fetal period. There are 83 cases of the disease reported
in English literature up to now. It was first described in the 2002 edition of WHO in the
group of soft tissue tumors. It is a rare tumor consisting of epithelial areas, spindle cells and
mature fat tissue. It is most commonly seen in middle-aged men. And is most commonly
seen in the supraclavicular, suprasternal and sternoclavicular areas. Ectopic tumors are
usually benign, but they can rarely cause local invasion and metastasis. According to the
WHO classification, thymic neoplasias are histopathologically divided into three types. Type
a, ''atrophic thymus'' representing thymic cells in adult life; type b, ''bioactive'' representing
the biologically active thymus of the fetus; Type C originates from the first letter of the term
"carcinoma". Type a thymoma corresponds to spindle cell and medullary thymoma. They
consist of neoplastic spindle-shaped epithelial cells without lymphocytes. It is a type AB
mixed tumor. It consists of a mixture of lymphocyte-poor type A and lymphocyte-rich type B
thymoma. Type B thymomas resemble normal thymus and contain plump epithelial cells.
They are divided into 3 subtypes according to the epithelial cell/lymphocyte ratio and atypia.
Type B1 thymoma; predominantly between epithelial cells mimicking the normal thymic
cortex structure. It contains distinct immature lymphocytes that are dispersed and may or
may not have a Hassal corpuscle. Type B2 thymoma; are neoplastic cells arranged in a large,
polygonal loose network. Type B3 thymoma (formerly classified as well-differentiated thymic
carcinoma) contains predominantly medium-sized round or polygonal cells with mild atypia.
These tumors are more aggressive than B1 and B2 thymoma. Metastasis and recurrence are
not uncommon. Type C thymoma accounts for <10% of thymic tumors. There are different
histological subtypes in this group: keratinizing and nonkeratinizing squamous cell
carcinoma, mucoepidermoid, basaloid, lymphoepithelioma-like, small cell/neuroendocrine,
sarcomatoid, clear cell and undifferentiated/anaplastic (3).
The staging system is used published by Masaoka et al and modified by Koga et al. This
classification system is based on local invasion, distant intrathoracic and extrathoracic
metastasis. In stage I, there is no macroscopic or microscopic capsule invasion. While there is
microscopic transcapsular invasion in stage 2A, there is macroscopically surrounding
mediastinal fatty tissue invasion in stage 2B. There is adjacent organ invasion in Stage 3,
pleural or pericardial spread in Stage 4A, and lymphogenous and hematogenous metastasis
in Stage 4B.
Staging and histology are the most important prognostic factors for thymic tumors, despite
all previously described limitations; however, additional prognostic factors have been
identified over time (1). Complete surgery is an important prognostic factor; Even in
advanced stage patients, the risk of recurrence is lower after complete resection (4). Tumor
size is another important prognostic factor (5). In previous years, the combination of
thymoma and MG was believed to be a poor prognostic factor. However, it was later
determined that the presence of MG was not a bad prognostic factor and survival was
significantly better in the MG group (6). This is probably because more patients with MG are
diagnosed at stages 1 and 2. Association with other paraneoplastic syndromes adversely
affects the prognosis, for example, acquired hypogammaglobulinemia and daf red cell
aplasia. Large vessel involvement (stage 3) is an independent prognostic factor (7).
Recurrence is stated as a poor prognostic factor (8).
Surgical resection is the recommended treatment approach in ectopic cervical thymoma.
Complete resection is the gold standard treatment to ensure complete cure. Stage 1 tumors
do not require additional treatment after complete resection. Stage 2B lesions, especially B2,
B3 and C tumors, have an increased risk of recurrence . Haniuda et al reported that RT will
provide an additional benefit in the lesion at this stage, even if there is no microscopic
pleural invasion. While the recurrence rate was 0% in those who received adjuvant RT, this
rate was shown to be 36.4% in those who did not receive RT (9). Strong evidence has been
shown for the need for postoperative RT in stages 3 and 4.

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