Fig 4 - uploaded by Ljubica Pejakov
Content may be subject to copyright.
Source publication
Introduction:
Thyroid paraganglioma is a very rare malignant neuroendocrine tumor. Immunohistochemical features of thyroid paraganglioma are helpful for the diagnosis.
Case report:
A 69-year-old female came to hospital with the presence of a growing thyroid nodule of the left lobe. Ultrasonic neck examination showed 5 cm hypoechoic nodule in the...
Similar publications
This study aimed at assessing the performance of 177Lu-DOTATATE-based peptide receptor radionuclide therapy (PRRT) in de-differentiated thyroid carcinoma thyroglobulin-elevated negative iodine scintigraphy (TENIS) in terms of clinical efficacy and outcome. This is a retrospective analysis of patients of TENIS who had undergone PRRT in a tertiary ca...
Citations
... 30,62 One patient underwent adjuvant peptide receptor radionuclide therapy after surgical removal of a locally aggressive thyroid paraganglioma without distant metastases, and no evidence of recurrent disease upon 3 years of follow-up was observed. 64 Most thyroid solitary fibrous tumours are benign and are surgically cured, with recurrent and metastatic disease being reported only in two patients. 12 Similarly, almost all reported thyroid granular cell tumours were benign, with locally aggressive disease reported in one patient who was eventually cured with surgery. ...
Most primary thyroid tumours are of epithelial origin. Primary thyroid mesenchymal tumours are rare but are being increasingly detected. A vast majority of thyroid mesenchymal tumours occur between the fourth and seventh decades of life, presenting as progressively enlarging thyroid nodules that often yield non-diagnostic results or spindle cells on fine needle aspiration biopsy. Surgery is the preferred mode of treatment, with adjuvant chemoradiotherapy used for malignant thyroid mesenchymal tumours. Benign thyroid mesenchymal tumours have excellent prognosis, whereas the outcome of malignant thyroid mesenchymal tumours is variable. Each thyroid mesenchymal tumour is characterised by its unique histopathology and immunohistochemistry. Because of the rarity and aggressive nature of malignant thyroid mesenchymal tumours, a multidisciplinary team-based approach should ideally be used in the management of these tumours. Comprehensive guidelines on the management of thyroid mesenchymal tumours are currently lacking. In this Review, we provide a detailed description of thyroid mesenchymal tumours, their clinical characteristics and tumour behaviour, and provide recommendations for the optimal management of these tumours.
... Although local extension is not regarded as a malignant feature in PTPGs [5], the frequency of this has perhaps been underestimated in the past. Eight cases report extension into the trachea or larynx [4,5,[17][18][19][20]39], with three invading the oesophagus [2,5,19] and three invading the recurrent laryngeal nerve [2,8,38]. Interestingly, three case reports describe vascular invasion by the tumour [4,38]. ...
Paragangliomas (PG) are very rare neuroendocrine tumours, arising from neural crest derived paraganglia of the autonomic nervous system. Primary thyroid paraganglioma (PTPG) is a rare site of PG and only 45 cases have been previously reported. The preoperative diagnosis of PTPGs presents a challenge as the clinical, cytological and histological features overlap with more common primary thyroid cancers. A 55 year old male was found to have significant enlargement of the left lobe of his thyroid. Following lobectomy, the thyroid lobe showed unencapsulated tumour which was positive for synaptophysin, CD56 and S100 (sustentacular cells). Post-operative imaging demonstrated incomplete resection. There was no post-operative radiotherapy and monitoring was by 6-12 monthly MRI. 48 months after his surgery he is alive and well with no evidence of disease progression. The diagnosis of PTPG was only made postoperatively, and although rare should be considered in the differential diagnosis of a hypervascular thyroid nodule.
Thyroid paragangliomas are distinctly rare primary thyroid neoplasms with nearly 75 cases reported worldwide. Due to their similar embryological origin and cytohistomorphology with other thyroid neoplasms, they may pose great diagnostic challenges for pathologists, radiologists, endocrinologists, as well as surgeons leading to unnecessary aggressive therapy. With recent advances in molecular genetics, the prognostic significance of such seemingly innocuous thyroid neoplasms has been better understood. In this manuscript, we describe such a case and present a systematic review of all reported cases till date giving an update on our current knowledge regarding their diagnostic pitfalls, pathology, and molecular genetics.
BACKGROUND
Pigmented paraganglioma is a special type of paraganglioma, and it is rare in the thyroid.
CASE PRESENTATION
We report a case of a 41-year-old woman who had complained of a mass in the thyroid gland. Histology revealed tumor cells arranged in a nest-like or organoid pattern, separated by delicate fibrovascular septa. Two distinct components were observed. In the first, which constituted the majority of the tumor cells, no pigments were observed. In the second, a few cells with pigment showed intercellular substance, but the structure was unclear. Using immunohistochemistry, cells in the first component were confirmed to be diffuse strong positive for synaptophysin, but negative for chromogranin A, pan-cytokeratin, calcitonin, and thyroglobulin. About 1% of tumor cells were stained by Ki-67. In the margins of the tumor, a few cells were observed to be positive for HMB-45 and Melan A after bleaching by oxalic acid. The stromal cells were positive for S-100. Using electron microscopy, a few cells containing many round melanin bodies with greater electron density granules of nonuniform size were observed. The diagnosis of primary melanotic paraganglioma of the thyroid gland was made.
CONCLUSION
Primary melanotic paraganglioma of the thyroid gland is a rare, low malignant potential tumor. To the best of our knowledge, this is the first case described.
Introduction:
Primary paraganglioma (PG) of the thyroid gland is an extremely rare neuroendocrine tumor with potential for misdiagnosis. We describe 2 cases of thyroid PG, suggest a possible diagnostic and therapeutic management strategy, and present a systematic review of the literature.
Case reports:
Two 67-year-old women presented similarly with asymptomatic but rapidly growing thyroid nodules in which malignancy was suspected after fine needle aspiration biopsy, "THY 4" according to the 2014 SIAPEC classification, both undergoing total thyroidectomy. Unexpectedly, immunohistochemistry showed neuroendocrine cellular architecture that was negative for common markers of well-differentiated follicular neoplasms, thyroglobulin, thyroid transcription factor 1, cytokeratins and medullary thyroid cancer, calcitonin, carcinoembryonic antigen, whereas neuron-specific enolase, synaptophysin, chromogranin A, and S-100 protein were highly expressed, confirming the diagnosis of primary thyroid PG. The patients were both discharged on postoperative day 2, without any other therapy and are currently well without evidence of local recurrence of metastatic disease, after 4 years and 3 months of follow-up, respectively.
Discussion:
These are the only 2 cases of thyroid PG experienced in our center which specializes in thyroid surgery. Thyroid PG is a rare neuroendocrine neoplasm first described by Van Miert in 1964 with just over 50 cases reported in the literature. Our experience is concordant with the literature that the diagnosis of the primary PG of the thyroid is challenging, due to its low prevalence and the cytologic and histopathologic similarities with other more frequently diagnosed benign and malignant thyroid tumors. Immunohistochemistry is required for definitive diagnosis but gross tumor characteristics are also helpful for diagnosis. Surgical resection is the recommended standard treatment.
