HISTOPATHOLOGY INDIA.COM Myxoid Tumours of Soft Tissue


Medullary carcinoma can appear in many different growth patterns.

The most common variant comprises small packets of short spindle

cells  with a readily recognizable "neuroendocrine" appearance.

It some rare cases papillary structures are present. 

Although papillae are present, these tumours are clearly not

papillary carcinoma because  the characteristic nuclear

changes are absent.

The confirmation of medullary carcinoma rests on argyrophil

stains, immunohistochemistry for calcitonin, calcitonin gene

product, other  neuroendocrine markers, and, somewhat

surprisingly, CEA, staining for  which is almost always strongly present.

Amyloid is present in about 50% of cases and its presence is

 said by some  to be associated with an indolent course.

Non-tumoural thyroid tissue should be scrutinised for C-cell

hyperplasia to  assist in determining whether the case is likely

to be familial.


Diagnosis of medullary thyroid carcinoma by calcitonin measurement in fine-needle aspiration biopsy specimens.Thyroid. 2007 Jul;17(7):635-8.

Medullary thyroid carcinoma (MTC) is a thyroid malignancy originating from C cells. To date, serum calcitonin measurement and fine-needle aspiration biopsy (FNAB) have been prominent diagnostic approaches to these lesions. Although an elevated serum calcitonin level strongly suggests the presence of MTC, this examination cannot identify the origin of the calcitonin overexpression, especially in patients demonstrating multiple thyroid nodules. For the treatment planning, it is important to know which nodule is MTC, especially in nonhereditary MTC. In this study, we propose calcitonin measurement in the washout of FNAB needles after sampling each tumor (FNAB-CT) as a new approach to diagnosis of MTC. We performed FNAB-CT for five MTC cases. Although only one of these cases was definitively diagnosed as having MTC by FNAB cytology, FNAB-CT values ranged from 17,000 to 560,000 pg/mL, which were exceedingly higher than those of 11 controls (seven papillary carcinomas, two adenomatous nodules, one chronic thyroiditis, and one normal thyroid), which showed values ranging from <10 to 67 pg/mL. In patients with MTC, FNAB-CT values were 74 to 1888 times greater than serum calcitonin values. These findings suggest that FNAB-CT can be an additional approach to diagnosis of MTC.

Molecular characterization of the desmoplastic tumor stroma in medullary thyroid carcinoma.Int J Oncol. 2007 Jul;31(1):59-67.

Medullary thyroid carcinomas are aggressive neoplasias that metastasize very early to loco-regional lymph nodes, and tumors with a desmoplastic stromal reaction have a higher incidence of lymph node metastasis. In order to characterize the desmoplastic response in thyroid cancers, we evaluated the expression pattern of three molecular markers of activated fibroblasts/myofibroblasts, namely, fibroblast activation protein alpha (FAPalpha), tenascin-C (Tn-C), and alpha-smooth muscle actin (alpha-SMA), as well as the endothelial markers endoglyx-1, CD34 and CD31 in a series of 28 metastatic and non-metastatic medullary thyroid cancers. Immunohistochemical studies demonstrated that the three fibroblast activation markers (FAPalpha, Tn-C, alpha-SMA) are consistently expressed in the peritumoral and intratumoral stromal compartment of medullary thyroid carcinomas and expression of FAPalpha and Tn-C correlated with the degree of desmoplasia determined histologically (p=0.001 for FAPalpha and p<0.001 for Tn-C). Moreover, the extent of desmoplasia as well as the expression of FAPalpha and Tn-C correlated with the presence of lymph node (LN) metastases (p=0.002, p=0.005 and p=0.002, respectively). No correlation was found between the microvessel density (neoangiogenesis) in the tumor stroma, assessed with the endoglyx-1, CD34 and CD31 markers, and the degree of desmoplasia or incidence of LN metastases. Using a bioinformatics-based search of the BioExpresstrade mark database we found in a series of 48 thyroid cancers a significant correlation between FAPalpha RNA expression and incidence of LN metastases also in papillary cancers. These findings suggest that the link between specific molecular markers of tumor stromal reaction and locoregional metastasis extends from medullary to other thyroid cancer types.

Current approaches to medullary thyroid carcinoma, sporadic and familial.J Surg Oncol. 2006 Dec 15;94(8):737-47.

Medullary thyroid carcinoma (MTC) is a rare malignancy of the thyroid C cells. It occurs in hereditary (25% of cases) and sporadic forms, and aggressiveness is related to the clinical presentation (hereditary vs. sporadic) and the type of RET mutation present. In hereditary cases, early diagnosis makes preventative surgery possible. In established cases, thorough surgical extirpation of the primary tumor and nodal metastases has been the mainstay of treatment. Radioactive iodine, external beam radiation therapy (EBRT), and conventional chemotherapy have not been effective. Newer systemic treatments, with agents that target abnormal RET proteins, hold promise and are being tested in clinical trials for patients with metastatic disease.

Natural history, diagnosis, treatment and outcome of medullary thyroid cancer: 37 years experience on 157 patients. Eur J Surg Oncol. 2007 May;33(4):493-7.

AIM: The analysis of a 37-year retrospective study on diagnosis, prognostic variables, treatment and outcome of a large group of medullary thyroid cancer (MTC) patients was conducted, in order to plan a possible evidence-based management process. METHODS: Between Jan 1967 to Dec 2004, 157 consecutive MTC patients underwent surgery in our centre: 60 males and 97 females, mean age 47.3 years (range 6-79). Total thyroidectomy was performed in 143 patients (91.1%); central compartment (CC) node dissection (level VI) in 41 patients; central plus lateral compartment (LC) node dissection (levels II, III, and IV) in 82 patients. Subtotal thyroidectomy was initially performed in 14 cases: 10 of them were re-operated because of persistence of elevated serum calcitonin levels. RESULTS: After a median post-surgical follow-up of 68 months (range 2-440 months), 42.9% of patients were living disease-free, 39.8% were living with disease, 3.1% were deceased due to causes different from MTC, and 3.2% were deceased due to MTC. The overall 10-year survival rate was 72%. At uni-variate statistical analysis (a) patient's age at initial treatment (>45 years; >/=45 years), (b) sporadic vs. hereditary MTC, (c) disease stage, and (d) the extent of surgical approach resulted as significant variables. Instead, at multivariate statistical analysis, only (a) patient's age at initial diagnosis, (b) disease stage, and (c) the extent of surgery resulted as significant and independent prognostic variables influencing survival. CONCLUSION: The presence of lymph node and distant metastases at first diagnosis significantly worsened prognosis and survival rate in our series. Early diagnosis of MTC is very important, allowing complete surgical cure in Stages I and II patients. Due to the relatively bad prognosis of MTC, especially for disease Stages III and IV, it appears reasonable to recommend radical surgery including total thyroidectomy plus CC lymphoadenectomy as the treatment of choice, plus LC lymphoadenectomy in patients with palpable and/or ultrasound enlarged neck lymph nodes.

May 2009


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Pseudoangiosarcomatous features in medullary thyroid carcinoma spindle-cell variant. Report of a case studied by FNA and immunohistochemistry.
Diagn Cytopathol. 2007 Jul;35(7):424-8.

We report a case of medullary thyroid carcinoma (MTC), spindle cell variant, which exhibited striking histological pseudovascular clefts and abortive lumina, closely mimicking an angiosarcoma. The patient is a 72-yr-old-man who presented facial rash and a solid nodule in the right lobe of the thyroid gland. The fine-needle aspiration (FNA) showed bloody background containing loosely groups of fusiform and plasmocytoid cells with coarse chromatin and eosinophilic granular cytoplasms. Microscopically, the tumor exhibited spindle-cell pattern intermingled with a striking angiosarcoma-like pattern characterized by the presence of abortive lumen and clefts containing erythrocytes. Dense hyaline extracellular amyloid was present. The tumor cells were strongly positive for cytokeratin, chromogranine-A, synaptophysine, serotonin, calcitonin, and CEA. TTF-1 was weakly positive. Stains for CD34 and CD31 were negative. This case illustrates that the spindle cell variant of MTC may exhibit an infrequent angiosarcoma-like appearance which may be misdiagnosed as angiosarcoma.

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