Variant Of Medullary Thyroid Carcinoma Research Articles

A case report of spindle cell variant of medullary thyroid carcinoma in Hashimoto’s thyroiditis

Background: Hashimoto’s thyroiditis is a common autoimmune thyroid disease and often coexists with differentiated thyroid carcinomas, but the link between medullary thyroid carcinoma and Hashimoto’s thyroiditis is not established, so the cases of these diseases occurring simultaneously are rare in the literature. Here we report a case of spindle cell variant of the medullary thyroid carcinoma in underlying Hashimoto’s thyroiditis. Case presentation: A 65-year old female was followed up in our Center because of hypothyroidism due to thyroid autoimmune disease. On neck ultrasound, an 11 X 7 X 9 mm hypoechoic nodule in the left thyroid lobe was found, and cytology was suggestive for spindle cell variant of medullary thyroid carcinoma. Calcitonin serum level was measured, and found elevated. After total thyroidectomy, pathology was compatible with spindle cell medullary thyroid carcinoma and Hashimoto’s thyroiditis. Conclusion: Medullary thyroid carcinoma in Hashimoto’s thyroiditis is rare, but must not be taken out of consideration when thinking about routine ultrasound check-ups in patients with non-nodular autoimmune thyroid disorder.

Spindle cell variant of medullary thyroid carcinoma: a clinicopathologic study of four cases

BackgroundMedullary thyroid carcinoma (MTC) is a malignant tumor derived from C cells. It accounts for about 10% of all thyroid malignancies. More than 14 histological variants have been described. Among them, spindle cell variant is extremely rare.Case presentationHere we describe 4 cases of spindle cell variant of MTC collected from 2012 to 2019. Ultrasound showed solid and hypoechoic nodules. Three patients underwent total thyroidectomy and regional lymph node dissection, and 1 patient underwent thyroid mass resection. Histologically, the tumors showed spindle shaped cells in bundles or interlaced arrangement, separated by hyalinised fibrous stroma that contained amyloid deposits. Immunohistochemistry showed that the tumor cells were positive for calcitonin, chromogranin A, synaptophysin, CD56, and TTF-1, but negative for other lineage-specific markers.ConclusionsWe report 4 rare cases of spindle cell variant of MTC. Due to its rarity and special morphology, the diagnosis of spindle cell variant MTC relies on its morphology and immunohistochemical markers to avoid misdiagnosis.

Spindle cell variant of medullary thyroid carcinoma: a clinicopathological analysis of 4 cases

目的： 探讨梭形细胞型髓样甲状腺癌（MTC）临床病理学特征。 方法： 收集2012至2019年空军军医大学（第四军医大学）西京医院诊断的梭形细胞型MTC 4例，分析其临床资料、病理学形态及免疫表型，并复习相关文献。 结果： 患者男性2例，女性2例，年龄26~54岁，患者均以颈部肿块就诊，其中1例有淋巴结转移。组织学观察，瘤组织以梭形细胞成分为主，细胞呈束状或交织状排列，细胞核呈短梭形或长梭形，胞质丰富，核仁不明显，核分裂象少见，肿瘤组织有纤维血管间质分隔伴淀粉样变，与周围甲状腺组织边界较清。免疫组织化学显示，所有病例肿瘤细胞均为降钙素、嗜铬粒素A（CgA）、突触素、CD56、甲状腺转录因子1（TTF1）阳性，Ki-67阳性指数3%~10%，而其他定向分化标志物均阴性。随访7~84个月，4例均健在，未见复发。 结论： 梭形细胞型MTC是一种少见MTC病理类型，诊断需要结合病理形态及免疫组织化学标记。.

Role of cytology in differentiating anaplastic thyroid carcinoma with osteoclast like giant cells from giant cell variant of medullary thyroid carcinoma

Anaplastic thyroid carcinoma (ATC) is a highly aggressive tumor and constitutes 2%–5% of all thyroid carcinomas. The cytological diagnosis of osteoclast giant cell variant of ATC is extremely rare, and only a few cases have been described in literature. A 67 - year-old female underwent fine-needle aspiration cytology from the thyroid nodule. Smears revealed hypercellular aspirate comprising of clusters and singly scattered atypical cells with few interspersed multinucleated tumor cells. Cytomorphological differential diagnosis of giant cell/pleomorphic variant of medullary carcinoma and anaplastic carcinoma was offered. Histomorphological features on cell block assisted with immunohistochemistry confirmed the diagnosis of ATC with osteoclastic-like giant cells. Computed tomography-guided right lung lesion aspirate was reported as metastasis of high-grade poorly differentiated carcinoma. The patient was managed with cisplatin-based chemotherapy. This case highlights the importance of cytological diagnosis of ATC to guide the treatment modality of chemotherapy thus avoiding surgery, especially in old moribund patient with lung metastasis.

Concomitant medullary thyroid carcinoma with paraganglioma-like pattern and papillary thyroid carcinoma.

SummaryA 74-year-old man was referred to the Endocrinology Unit because of multinodular goiter. The dominant nodule (1.7 × 1.9 × 2.4 cm), at the medium-superior third of the left lobe, was inhomogeneously hypoechoic, with irregular margins, macrocalcifications and intranodular vascularization. Fine-needle aspiration biopsy (FNAB) was performed. The cytological diagnosis was TIR 2, benign, according to the 2013 Italian thyroid cytology classification system. Moderately high serum calcitonin (s-Ct) (61.5 pg/mL, n.r. 0–7.5) and normal CEA were detected. The Ct level in FNAB wash-out fluid (Ct-FNAB) was 1450 pg/mL. Based on s-Ct and Ct-FNAB levels, patient underwent total thyroidectomy. Macroscopically, a dominant circumscribed nodule of 2 ecm was described; the histological and immunohistochemical features identified medullary thyroid carcinoma (MTC) with paraganglioma (PG)-like pattern positive for Ct, CEA and chromogranin and negative for S-100 sustentacular cells (SC). Moreover, papillary carcinoma of 3 mm in the right lobe was also associated. No areas of hyperaccumulation of the tracer were documented at Ga68 PET/CT. No RET-proto-oncogene mutations were found. Post-surgery s-Ct levels were within normal range (4 pg/mL). Two years after thyroidectomy, the patient is still disease-free. We reported a case of sporadic and rare variant of MTC: this is the ninth described case of PG-like MTC. In this case, cytologically benign, the clinical suspicion arose from high Ct values at FNAB wash-out fluid. Even if clinical behavior of this variant seems indolent, additional studies are necessary to understand prognoses and predictive factors.Learning points:Several unusual histological variants of medullary thyroid carcinoma (MTC) have been described such as spindle cell, giant cell, clear cell, melanotic, squamous, angiosarcoma-like variants; even rarer is the paraganglioma (PG)-like pattern.We here describe a case of medullary PG-like thyroid carcinoma in a 74-year-old man. This is a rare histological variant of MTC hardly diagnosed by cytology, since immunohistochemical investigations are necessary.Measurement of calcitonin both in serum and in wash-out fluid from fine-needle aspiration could be an additional tool for an early and non-invasive identification of these variants.

Spindle epithelial tumor with thymus-like differentiation (SETTLE): Case report with longest follow up & latency to metastasis

Spindle epithelial tumor with thymus-like differentiation (SETTLE) is an extremely rare, low-grade malignant spindle cell tumor of the thyroid gland with delayed metastatic potential. It occurs mainly in children, adolescents and young adults and is thought to be derived from thymic or branchial pouch remnants. Nearly 42 cases have been reported in the English literature. Metastatic disease has been described up to 22 and 25years after the initial operative intervention. SETTLE should be considered in the differential diagnosis of any spindle cell tumor in thyroid region. Pathologic diagnosis requires spindle and epithelial components and the use of immunohistochemistry. The differential diagnosis is broad and includes synovial sarcoma, spindle cell anaplastic thyroid carcinoma, spindle cell variant of medullary thyroid carcinoma, thymoma, fibrosarcoma, leiomyoma and schwannoma. Owing to delayed metastasis, close long term follow-up is mandatory. We present a case where metastatic disease appeared in the lungs 35years after the initial diagnosis, representing the longest reported latency period to metastasis, which unfortunately led to the patient's demise.

Diagnosis of pseudopapillary variant of medullary thyroid carcinoma by fine‐needle aspiration cytology

Diagnostic CytopathologyVolume 42, Issue 9 p. 823-826 Letter to the Editor Diagnosis of pseudopapillary variant of medullary thyroid carcinoma by fine-needle aspiration cytology Namiki Kawanishi C.M.I.A.C., Namiki Kawanishi C.M.I.A.C. Department of Clinical Laboratory, Mihara Medical Associations Hospital, Hiroshima, JapanSearch for more papers by this authorYoshiaki Norimatsu Ph.D., C.F.I.A.C., Yoshiaki Norimatsu Ph.D., C.F.I.A.C. Department of Medical Technology, Ehime Prefectural University of Health Sciences, Ehime, JapanSearch for more papers by this authorHiroyuki Ohsaki Ph.D., C.F.I.A.C., Hiroyuki Ohsaki Ph.D., C.F.I.A.C. Department of Medical Technology, Ehime Prefectural University of Health Sciences, Ehime, JapanSearch for more papers by this authorTsutomu Yuminamochi C.T., C.M.I.A.C., Tsutomu Yuminamochi C.T., C.M.I.A.C. Department of Pathology, University of Yamanashi Hospital, Yamanashi, JapanSearch for more papers by this authorRyohei Katoh M.D., Ph.D., Ryohei Katoh M.D., Ph.D. Department of Pathology, University of Yamanashi Hospital, Yamanashi, JapanSearch for more papers by this authorKen Okusaki M.D., Ph.D., Ken Okusaki M.D., Ph.D. Department of Internal Medicine, Mihara Medical Association Hospital, Hiroshima, JapanSearch for more papers by this authorYukio Sato M.D., Ph.D., Yukio Sato M.D., Ph.D. Department of Surgery, Mihara Medical Association Hospital, Hiroshima, JapanSearch for more papers by this authorTadao K. Kobayashi Ph.D., C.F.I.A.C., Corresponding Author Tadao K. Kobayashi Ph.D., C.F.I.A.C. Education and Research Center, Osaka University Graduate School of Medicine and Health Science, Osaka, JapanCorrespondence to: Tadao K. Kobayashi, Ph.D., C.F.I.A.C., Education and Research Center, Osaka University Graduate School of Medicine and Health Science, Osaka, Japan, E-mail: [email protected]Search for more papers by this author Namiki Kawanishi C.M.I.A.C., Namiki Kawanishi C.M.I.A.C. Department of Clinical Laboratory, Mihara Medical Associations Hospital, Hiroshima, JapanSearch for more papers by this authorYoshiaki Norimatsu Ph.D., C.F.I.A.C., Yoshiaki Norimatsu Ph.D., C.F.I.A.C. Department of Medical Technology, Ehime Prefectural University of Health Sciences, Ehime, JapanSearch for more papers by this authorHiroyuki Ohsaki Ph.D., C.F.I.A.C., Hiroyuki Ohsaki Ph.D., C.F.I.A.C. Department of Medical Technology, Ehime Prefectural University of Health Sciences, Ehime, JapanSearch for more papers by this authorTsutomu Yuminamochi C.T., C.M.I.A.C., Tsutomu Yuminamochi C.T., C.M.I.A.C. Department of Pathology, University of Yamanashi Hospital, Yamanashi, JapanSearch for more papers by this authorRyohei Katoh M.D., Ph.D., Ryohei Katoh M.D., Ph.D. Department of Pathology, University of Yamanashi Hospital, Yamanashi, JapanSearch for more papers by this authorKen Okusaki M.D., Ph.D., Ken Okusaki M.D., Ph.D. Department of Internal Medicine, Mihara Medical Association Hospital, Hiroshima, JapanSearch for more papers by this authorYukio Sato M.D., Ph.D., Yukio Sato M.D., Ph.D. Department of Surgery, Mihara Medical Association Hospital, Hiroshima, JapanSearch for more papers by this authorTadao K. Kobayashi Ph.D., C.F.I.A.C., Corresponding Author Tadao K. Kobayashi Ph.D., C.F.I.A.C. Education and Research Center, Osaka University Graduate School of Medicine and Health Science, Osaka, JapanCorrespondence to: Tadao K. Kobayashi, Ph.D., C.F.I.A.C., Education and Research Center, Osaka University Graduate School of Medicine and Health Science, Osaka, Japan, E-mail: [email protected]Search for more papers by this author First published: 27 July 2013 https://doi.org/10.1002/dc.23020Citations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Citing Literature Volume42, Issue9September 2014Pages 823-826 RelatedInformation

Leiomyosarcoma: A Rare Tumor of the Thyroid

Primary leiomyosarcoma of the thyroid gland is uncommon. To date, 20 cases have been reported in English in the literature. The tumors usually present in elderly patients with female predilection and are associated with poor clinical outcome. Herein, we report an additional case of primary thyroid leiomyosarcoma in a 64-year-old woman. She underwent total thyroidectomy and later was discovered to have multiple lung and liver metastases. The patient died 3 months after surgery. The major differential diagnoses including undifferentiated (anaplastic) carcinoma of the thyroid, spindle cell variant of medullary thyroid carcinoma, spindle cell tumor with thymus-like differentiation, uncommon primary tumor of the thyroid and metastatic tumors with predominant spindle cells are discussed.

Melanocytic Variant of Medullary Thyroid Carcinoma in a Previously Treated Papillary Carcinoma Patient

Melanocytic Variant of Medullary Thyroid Carcinoma in a Previously Treated Papillary Carcinoma Patient

Malignant Peripheral Nerve Sheath Tumour of the Breast in Complex Clinical Background

Malignant Peripheral Nerve Sheath Tumour of the Breast in Complex Clinical Background Here we describe a patient presenting with combination of three rare diseases, namely de novo malignant peripheral nerve sheath tumour of the breast, small cell variant of medullary thyroid carcinoma and multiple sclerosis. Although the combination by chance seems unlikely, the detailed history does not correspond to any of the known hereditary cancer syndromes and hypothetically may represent a new entity.

Paraganglioma‐like medullary thyroid carcinoma: fine needle aspiration cytology features with histological correlation

Two cases of an extremely rare paraganglioma-like variant of medullary thyroid carcinoma (MTC) are reported. The patients were a 65-year-old male (case 1) and a 14-year-old female (case 2). Unilateral thyroid nodule and homolateral cervical lymphadenopathy was present in case 1; bilateral thyroid nodules were seen in case 2. Fine needle aspiration cytology (FNAC) was performed from thyroid nodules (in both cases) and from a cervical lymph node (in case 1). The cytological smears contained predominantly ovoid to spindled epithelial cells arranged in cohesive three-dimensional clusters with sharp margins; isolated individual cells were seen only rarely. No colloid or other material was present in the background. The tumour cells showed significant nuclear atypia with occasional bizarre and/or binucleated cells. The nuclear chromatin was coarse and granular, sometimes with grooves and intranuclear inclusions. The cytoplasm was inconspicuous. Polygonal or triangular cells, amyloid and azurophillic cytoplasmic granules were absent in both cases. Calcitonin expression was demonstrated in case 2. Histological examination confirmed the paraganglioma variant of MTC in both cases. Mutation of RET proto-oncogene in exon 16 (Met918Thr) - germline in case 2 and somatic in case 1 was detected by sequencing of DNA in both cases. This is the first description of cytological findings in the paraganglioma-like variant of MTC. Despite its rarity, it can be reliably diagnosed by FNAC if material for immunocytochemistry is obtained.

Metastatic Small Cell Carcinoma to the Thyroid Gland: a Pathologic and Molecular Study Demonstrating the Origin in the Urinary Bladder

Small cell carcinomas may occur in the thyroid gland. Infrequently, they are primary tumors, and have been interpreted as variants of medullary thyroid carcinoma. However, the vast majority of small cell carcinomas involving the thyroid gland are metastatic tumors. In some cases, demonstration of the primary tumor is not easy. An example of a small cell carcinoma metastatic to the thyroid is presented in this report. The primary tumor was a small cell carcinoma that occurred as a minor component in a transitional carcinoma of the urinary bladder. The microscopical and immunohistochemical features of both tumors, in the thyroid and the bladder, were identical. Moreover, both tumors exhibited an identical mutation in p53, as well as similar loss of heterozygosity at 10q23 and RASSF1A promoter hypermethylation, clearly indicating that the bladder tumor was the site for the primary tumor of the patient.

Paraganglioma-Like Medullary Thyroid Carcinoma: A Rare Entity

The wide variety of rare histologic variants of medullary thyroid carcinoma (MTC) may make the differential diagnosis difficult. Pathologic examination of the resected specimen will not always be able to confirm the diagnosis, leaving the surgeon with an uncertainty as to what type of resection is best for the patient. The following report describes the case of a 58-year-old man with the rare diagnosis of paraganglioma-like MTC. The patient presented with markedly elevated calcitonin (CT) and carcinoembryonic antigen (CEA) levels. A 1-cm tumor was detected in the right lobe of the thyroid. Based on the clinical diagnosis of MTC, we performed a total thyroidectomy with lymphadenectomy of the central and right lateral compartments. Paraffin sections of the resected specimen were stained with hematoxylin and eosin (H&E) and immunohistochemically characterized using antibodies to CT, CEA, chromogranin A, thyroglobulin, synaptophysin, sustentacular cells (SCs), low- and high-molecular cytokeratins (CK 5/6, 7, 18, 20), epidermal growth factor-receptor (EGFR), thyroid transcription factor-1 (TTF-1), bcl-2, Melan A, C-kit, neuron-specific enolase (NSE), and galectin-3. The patient's blood and tumor tissue were examined for mutations in the RET-protooncogene. H&E staining of both frozen and permanent sections was unable to differentiate benign from malignant tissue. Typical morphologic characteristics for MTC were completely absent. Only the additional finding of positivity for synaptophysin and numerous SC cells visible in-between neoplastic cells made the diagnosis of paraganglioma-like MTC possible. Sequencing of the RET proto-oncogene revealed no mutations. There are subgroups of MTC that present clinically similar to classic MTC, but in which missing typical morphologic characteristics make histopathology diagnosis difficult. In these cases, diagnosis, operative decisions, and follow-up strategies should be based on preoperative biochemical markers, imaging findings, and clinical parameters in accordance to the guidelines for classic MTC.

Clear cell variant of medullary carcinoma of the thyroid

A previously unreported clear cell variant of medullary thyroid carcinoma is described. This histologic appearance was observed in sections from both the primary thyroid tumor and a vertebral metastasis. This variant merits recognition because it may be confused with a variety of other tumors occurring in the thyroid that contain cells with optically clear cytoplasm. Immunohistochemical and ultrastructural studies can be helpful in establishing the diagnosis of these tumors.