Malignant Thyroid Teratoma with Aggressive Neck and Intrathoracic Disseminations

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1 中華癌醫會誌 (J. Chinese Oncol. Soc.) 25(6), , 2009 Case Report journal homepage: Malignant Thyroid Teratoma with Aggressive Neck and Intrathoracic Disseminations Wei-Chun Lo, Shi-Ping Luh,2 *, Dong-Long Wu, Mao-Te Chuang Department of Surgery, St Martin De Porres Hospital, Chia-Yi City, Taiwan 2 Institute of Biomedical Engineering, National Chiao-Tung University, Hsin-Chu City, Taiwan 病例報告 Abstract. Background: Primary malignant teratoma of the thyroid is extremely rare. There were only around 30 patients reported in the literature. Case Report: A 60-year-old housewife was found with a growing neck mass 3 months before first admission. Follicular carcinoma of the thyroid was told after surgical resection (Hartley Dunhill procedure). Several rapid growing masses over the supraclavicular and right lung were noted 6 months later. She underwent tumor resection. Rapid growing mass with tracheal and mediastinal compression was noted 3 months after operation, and debulking of the tumors was performed to secure her airway. Malignant teratoma was proved by pathological examination. Tracing back her history and clinical data, the patient had had elevated serum alpha-fetoprotein (α-fp) data since her first operation and no liver disease was found then. Reviewing her past pathological slides from the resected specimen, the diagnosis of malignant teratoma was confirmed. Conclusions: Malignant teratoma of the thyroid is extremely rare, but should be listed as a differential diagnosis in patients with neck masses. Pathological examination with immuno- histopathological staining can be used to confirm the diagnosis. This tumor is aggressive, with poor outcome. Surgical resection combined with adjuvant chemo-irradiation might be helpful to these patients. Keywords : thyroid, malignant teratoma, mediastinum 甲狀腺惡性畸胎瘤合併頸部及胸內轉移 羅偉鈞 陸希平,2 * 吳東龍 莊茂德 嘉義天主教聖馬爾定醫院外科部 2 國立交通大學生醫工程所 中文摘要原發性甲狀腺惡性畸胎瘤是非常罕見的疾病, 到目前為止文獻上有記載的病例大約有三十例, 其中大部份患者是女性, 年齡從八歲到五十六歲都有 今天這個病例報告是一位六十歲女性家庭主婦, 因為就醫前三個月頸部腫瘤不斷長大接受第一次手術, 病理報告為甲狀腺濾泡癌, 術後接受碘 -3 治療及甲狀腺素治療, 但療效不彰

2 470 W. C. Lo et al./jcos 25(2009) 六個月後, 因右側鎖骨上區及肺部快速長出腫塊, 病人接受第二次切除手術 不幸的是, 腫瘤仍快速長出, 三個月後出現在頸部與縱隔腔並壓迫呼吸道及附近血管, 病人接受手術切除減壓術 這時病理發現才懷疑是惡性畸胎瘤 病人從第一次就醫前幾個月就已在健康檢查中發現甲型胎兒球蛋白不正常的增高, 但並無發現肝內或其它部位的腫瘤 在比對過去數次的檢體及特殊染色後證實了惡性畸胎瘤的診斷 在甲狀腺腫瘤中, 惡性畸胎瘤雖然罕見, 但是必須列入考慮, 才不致於失去有效治療先機 病理診斷使用免疫化學染色可確定其診斷 惡性畸胎瘤侵襲性高, 預後不佳, 早期外科切除合併化電療可有效控制病情 關鍵字 : 甲狀腺 惡性畸胎瘤 縱隔腔 INTRODUCTION Teratomas comprise the most common extragonadal germ cell tumors in childhood []. Teratomas from the head and neck account for only about 5% of all germ cell tumors and for 6% of all teratomas, where the most common sites of involvement are the neck, thyroid, superficial facial structures, nasopharynx, and orbit [2,3]. Thyroid teratomas are mostly benign and occur in children. Malignant thyroid teratomas are extremely rare. There were only around 30 patients, mostly women with ages ranging from 8 to 56 years, reported in the literature [2-7]. Herein, a 60-year-old woman with primary malignant thyroid teratoma with subsequent regional and intrathoracic disseminations is reported. CASE REPORT A 60-year-old housewife presented to our clinic with a three-month history of a growing painless mass in the neck. She had been noted with hypertension and diabetes mellitus for about ten years with regular medical control. There were no other systemic diseases, and as well no drinking or smoking habits for her. On physical examination, a firm mass was noted on *Corresponding author: Shi-Ping Luh M.D., Ph. D. * 通訊作者 : 陸希平醫師 Tel: ext.003 Fax: luh57200@yahoo.com.tw the right side of her neck, over the thyroid area. No cervical lymph node enlargement or jugular vein engorgement was noted. A mass over the right lobe of the thyroid was noted on sonography, but examinations of specimens from this tumor by fine needle aspiration were negative for malignancy. Right thyroid lobectomy, followed by subtotal resection of the left lobe two weeks later was performed. This tumor, occupying the whole right lobe of the thyroid, was 7x 4 x 2 cm in size, and 40 gm in weight. Follicular carcinoma of thyroid was then diagnosed by histopathology. Radio-iodide (I-3) evaluation revealed no residual hot spots on extra-thyroid area, and I-3 ablation was administered post-operatively. The patient took oral thyroxin daily after completion of the above therapy. Unfortunately several rapidly growing masses over the right supraclavicular and right lung were noted 6 months later (Figure ). She underwent tumor resection at another hospital and only poorly differentiated carcinoma with unknown origin was reported then. Numerous rapidly growing masses with sizes up to 0 cm at the paratracheal, mediastinal, and lung parenchyma were were noted 3 months after operation (Figure 2). The radio-iodide evaluation revealed cold signal over the tumor sites. Debulking the tumors on the neck and mediastinum through right cervical curvilinear incision and median sternotomy was performed to secure the patient s airway. Malignant teratoma was proved by pathological examination. Tracing back her history and clinical data, she had had elevated α-fp data (from 5 to 50 ng/ml) since her

3 W. C. Lo et al./jcos 25(2009) Figure. Computed tomography revealing right supraclavicular (A) and lung (B) masses (arrow) Figure 2. Computed tomography revealing right cervical (A) and mediastinal (B) masses (arrow) first operation, and no liver disease was found then. beneath the skin with focal hemorrhage. Gross find- Reviewing her past pathological slides from the re- ings of the mediastinal mass showed a 2x7x6.5 cm sected thyroid specimen, the diagnosis of malignant mass en bloc with the invaded lung tissue 7.5x5x2.8 teratoma was confirmed. cm in size with a satellite nodule 2.4x2.2x.5 cm in The gross picture of the cervical specimen was size. Microscopically, these masses were found to be 6.x5.3x4.7 cm in size with an overlying skin meas- composed of two types of germ cell tumors, mainly uring 4.8x2.7 cm in area. On the cut surface, there was (70%) immature teratoma and minor (30%) seminoma. a well circumscribed tumor, 5.3x4.4x3.7 cm in size, The immature teratoma component revealed remarkable

4 472 W. C. Lo et al./jcos 25(2009) immature neural tissue forming tubes, focal immature cartilage and squamous epithelium. The seminoma component showed a confluent multinodular clusters and irregular lobules. The tumor cells revealed lightly eosinophilic cytoplasm and distinct cell membranes. Between the tumor cell aggregates, delicate fibrovascular septa were observed. There was focal extensive necrosis in the immature teratoma component. Vascular permeation was easily found. Immunohistochemi- A cal staining showed strongly positive for vimentin but only weakly positive for cytokeratin,chromogranin or SMA (Figure 3). DISCUSSION Teratomas usually occur in the ovaries, testes, paraaxial areas from the brain, mediastinum, and retroperitonium to the sacrum [2]. Teratomas of the thyroid mostly occur in children and are usually benign. Malignant thyroid teratoma, first reported in 908, is extremely rare. By 200, only 27 cases had been report- B ed in the medical literature [6]. Since then, only four new cases have been reported [3-5,7]. Malignant thyroid teratomas predominantly occur in females (male: female about :3) and after adolescence [6]. Only two children less than 0 years of age have been reported before [7,8]. The age of our presented case is older than any other reported cases in the literature. Confirming teratomas from the thyroid should exclude the possibilities of these tumors from other anterior cervical sites. Criteria that designate a teratoma of thyroid origin include that the tumor is present C within the gland, the thyroid is replaced by the tumor, Figure 3. (A) The pulmonary tissue shows malignant or the gland is continuous with the tumors [9]. The tumor cell nests infiltrate. The tumor cells tissue diagnosis of benign teratoma is determined by show small blue round cells with neuroen- observation of cellular structure from three germ cell docrine like features. (H&E stain, 20X). (B) layers. However, malignant teratoma manifests pre- It shows immature teratoma with immature dominantly with primitive neuroepithelium mixed neural tissue forming tubes (H&E stain with another tissue components. Accurate diagnosis is 0X). (C) There are immature squamous very difficult to establish by fine-needle biopsy, or epithelium and immature neural tubes. even small biopsy specimens. Furthermore, frozen- (H&E stain, 20X) section diagnosis of teratomas may be difficult, and

5 W. C. Lo et al./jcos 25(2009) therefore, thorough pathological sectioning and staining (including special staining) are required [0,]. In the present case, accurate diagnosis could not be confirmed at the time of the first and second operations for primary and metastatic tumors. However, reliable diagnosis could be established after checking the new and rechecking the prior resected specimens. There were two components (teratoma and seminoma) in the tumors of our presented case, and the immature teratoma was composed of predominantly immature neural tissue forming tubes, admixed with focal immature cartilage and squamous epithelium. Malignant thyroid teratomas are aggressive in nature. Such patients usually present with a rapidly growing mass on the neck, with subsequent respiratory or swallowing compromise from tracheal or esophageal compression by tumors. Metastases into the lung or cervical lymph nodes are not uncommon, and the medium survival is short (8 months) after surgery without postoperative adjuvant therapy [2,3]. Therefore, it is necessary for patients with malignant teratomas from the thyroid to combine chemotherapy with similar regimens to ones from other origins [4,2]. Chen et al reported a 32-year-old female who survived over 7 years after undergoing surgical therapy followed by chemotherapy with CDDP, VP-6 and Bleomycin, and as well local irradiation on local recurrence [3]. Kim et al. followed the rule of NCI protocol INT-009 which had been used in treating neuroectodermal tumors from other sites. They applied combination chemotherapy postoperatively with ifosfamide and etoposide in a 3-year-old woman with malignant teratomas of the thyroid with predominantly neuroectodermal components, and got at least two years relapse-free survival [3]. Some investigators have reported the role of radiotherapy for palliation of these patients with locoregional recurrences [4]. In the present case, since the confirmation of diagnosis was delayed, we could not administer treatment timely and effectively for her. In conclusion, malignant teratomas of the thyroid are extremely rare and predominantly occur in females after adolescence. Tissue diagnosis of these tumors requires thorough pathological evaluation. These tumors are usually aggressive, presenting with rapidly growing loco-regional recurrence or distant metastasis. Surgical resection followed by local irradiation or systemic chemotherapy are usually required to improve the survival of these patients. REFERENCES. Lack EE. Extragonadal germ cell tumors of the head and neck region: review of 6 cases. Hum Pathol 6: 56-64, Djalilian HR, Linzie B, Maisel RH. Malignant teratoma of the thyroid: review of literature and report of a case. Am J Otolaryngol 2: 2-5, Kim E, Bae TS, Kwon Y, et al. Primary malignant teratoma with a primitive neuroectodermal tumor component in thyroid gland: a case report. J Korean Med Sci 22: , Tsang RW, Brierley JD, Asa SL, et al. Malignant teratoma of the thyroid: aggressive chemoradiation therapy is required after surgery. Thyroid 3: , Martins T, Carrilho F, Gomes L, et al. Malignant teratoma of the thyroid: case report. Thyroid 6: 3-33, Craver RD, Lipscomb JT, Suskind D, et al. Malignant teratoma of the thyroid with primitive neuroepithelial and mesenchymal sarcomatous components. Ann Diagn Pathol 5: , Majhi U. Primary malignant teratoma of the thyroid in a child with nodal metastases. Indian J Pathol Microbiol 52: , Lecomte-Houcke M, Parent M, Carnaille B, et al. [Primary malignant teratoma of the thyroid. Two cases involving immunohistochemical and ultrastructural studies]. Ann Pathol 2: 2-9, Silberman R, Mendelson IR. Teratoma of the neck:

6 474 W. C. Lo et al./jcos 25(2009) report of two cases and review of the literature. Arch Dis Child 35: 59-70, Murao T, Nakanishi M, Toda K, et al. Malignant teratoma of the thyroid gland in an adolescent female. Acta Pathol Jpn 29: 09-7, Vujanic GM, Harach HR, Minic P, et al. Thyroid/cervical teratomas in children: immunohistochemical studies for specific thyroid epithelial cell markers. Pediatr Pathol 4: , Siegert W, Rick O, Beyer J. High-dose chemotherapy with autologous stem cell support in poor-risk germ cell tumors. Ann Hematol 76: 83-88, Chen JS, Lai GM, Hsueh S. Malignant thyroid teratoma of an adult: a long-term survival after chemotherapy. Am J Clin Oncol 2: 22-24, Als C, Laeng H, Cerny T, et al. Primary cervical malignant teratoma with a rib metastasis in an adult: five-year survival after surgery and chemotherapy. A case report with a review of the literature. Ann Oncol 9: , 998.