Cases reported "Carcinoma, Papillary"

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1/14. Scarring in papillary carcinoma of the thyroid: report of two new cases with exuberant nodular fasciitis-like stroma.

    AIMS: To describe two new cases of papillary carcinoma of the thyroid with exuberant nodular fasciitis-like stroma, one of which was characterized by previously unreported transformation into a poorly differentiated lesion. Moreover, we explore the presence of TGF-beta to help to clarify the pathogenesis of the collagen formation. methods AND RESULTS: The case characterized by an aggressive behaviour exhibited areas of transformation into a poorly differentiated (insular) carcinoma of the thyroid. In both cases, as revealed by immunohistochemistry, neoplastic cells produced and secreted high amounts of TGF-beta. On the contrary, TGF-beta immunoreaction was never present in the normal thyroid or in papillary carcinomas without collagen bundles, while a weak, exclusively intracellular reaction was present in a patchy manner in cases showing intratumoral fibrous bundles. CONCLUSIONS: The rare variant of papillary thyroid carcinoma characterized by exuberant stroma may give rise to more aggressive lesions, as do other histotypes of differentiated thyroid carcinomas. TGF-beta, the fundamental cytokine which mediates scarring and activation of myofibroblasts, most probably induces the exuberant stroma.
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2/14. Familial papillary carcinoma of the thyroid: a report of nine first-degree relatives of four families.

    The authors report the occurrence of papillary carcinoma of the thyroid in nine first-degree relatives of four families among a consecutive series of 97 patients with papillary carcinoma of the thyroid who were operated on from 1991 to 1998. Total thyroidectomy was performed in all cases. All patients are alive without evidence of disease after a mean follow-up period of 43 months. Since in our series familial papillary carcinoma of the thyroid was found in 9.3% of patients, we suggest an adequate screening among first-degree relatives of all patients with papillary thyroid carcinoma. Because of reported aggressive behaviour of familial papillary carcinoma of the thyroid, aggressive surgical treatment plus post-operative thyroid remnant ablation with radio-iodine should be warranted in all patients.
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3/14. Mixed columnar cell and tall cell variant of papillary carcinoma of thyroid: a case report and review of the literature.

    Columnar cell and tall cell carcinomas are newly described variants of papillary thyroid carcinoma associated with aggressive clinical behaviour. Although several cases of tall cell and columnar cell variants have been reported, only a single detailed case report of a mixed tall cell and columnar cell variant has been described in the English-language literature. We report another such composite tumour with predominant columna cell features in an elderly female. The tumour showed extrathyroidal extension with intraluminal superior thyroid vein invasion and lymph node metastasis. dna ploidy analysis showed a diploid dna content with no increase of S-phase fraction. immunohistochemistry showed focal positivity for p53 and Ki-67 at the infiltrating margins of the tumour and diffuse positivity for proliferating cell nuclear antigen. The adverse clinical course warrants aggressive treatment and careful follow-up.
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4/14. Aggressive behaviour of solid arrangement pattern in differentiated papillary carcinoma of thyroid.

    The prognosis and overall outcome of solid growth pattern in differentiated papillary carcinoma of thyroid is controversial. While general consensus suggest outcomes similar to typical papillary carcinomas others demonstrate a more aggressive biology. We present a case of differentiated papillary carcinoma of thyroid with solid growth pattern presenting with neck swelling and widespread skeletal metastases. The areas of bone lesions showed avid 131I concentration on a postoperative large dose 131I whole body scan and was treated with 131I subsequently. The present case suggest that the solid architecture in a papillary thyroid carcinoma may be indicative of an aggressive clinical course, contrary to the common opinion that it does not adversely influence its biologic behaviour and thus emphasize the need to reexplore the prognostic significance of histopathologic subclassification along with an assessment of histologic grade and expression of molecular risk factors in this particular tumour subtype.
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5/14. Columnar cell thyroid carcinoma - diagnostic dilemmas and pitfalls.

    BACKGROUND: Columnar cell carcinoma is a rare variant of papillary thyroid carcinoma associated with aggressive clinical behaviour. A CASE REPORT: of a 34-year-old male patient, who presented with the rapidly growing mass in the neck, extending to the anterior and middle mediastinum, tightly closing the upper thoracic apperture and causing tracheal and oesophageal deviation, with minimal compression and stenosis. A diagnosis of columnar cell carcinoma, arising from the ectopic thyroid tissue just adjacent to the left thyroid lobe was based on histological and intraoperative findings. Near total thyroidectomy and lymph-node dissection were followed by external beam radiotherapy of the neck and mediastinum, chemotherapy and radioiodine ablation of the remaining functional thyroid tissue. Pre-and post-operative radionuclide imaging (99mTc(V)-DMSA, 99mTc-MIBI, 123-I-mIBG and Octreoscan findings are discussed, with a special emphasis given to the dilemmas in histological characterisation of the tumor, the problems in therapeutic approach and the dilemmas and pitfalls in the interpretation of radionuclide findings in this patient, especially the ones performed post radiotherapy of the neck and mediastinum.
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6/14. Medullary and papillary carcinoma of the thyroid gland occurring as a collision tumour: report of three cases with molecular analysis and review of the literature.

    We report the simultaneous occurrence of medullary thyroid carcinoma (MTC) and papillary thyroid carcinoma (PTC), presenting as spatially distinct and well-defined tumour components, in three cases. In the first patient, histology, immunohistochemistry and electron microscopy demonstrated an MTC in the one nodule and PTC in two additional lesions. Non-neoplastic thyroid parenchyma separated the three nodules. Metastasis from PTC was diagnosed in a regional lymph node. Genetic analysis of both tumour components showed a distinctive mutational pattern: in the MTC a Cys634Arg substitution in exon 11 of the RET gene and in the two PTC foci a Val600Glu substitution in exon 15 of the BRAF gene. The other two patients are members of a large multigenerational family affected with familial MTC due to a germline mutation of the RET gene (Ala891Ser). Both patients harboured, besides medullary cancer and C-cell hyperplasia, distinct foci of papillary thyroid cancer, which was positive for Val600Glu BRAF mutation. review of the literature disclosed 18 similar lesions reported and allowed the identification of different patterns of clinical presentation and biological behaviour. So far, the pathogenesis of these peculiar cases of thyroid malignancy has been completely unknown, but an underlying common genetic drive has been hypothesised. This is the first report in which two mutations, in the RET and BRAF genes, have been identified in three cases of MTC/PTC collision tumour, thus documenting the different genetic origin of these two coexisting carcinomas.
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7/14. Metastatic solid-pseudopapillary tumour of the pancreas: clinico-biological correlates and management.

    Solid-pseudopapillary tumour of the pancreas is a rare neoplasm of young women, currently categorised in the world health organization classification under exocrine pancreatic tumours. Increased awareness of this condition correlated recently with an apparent rise in incidence as well as recognition of more aggressive clinical courses. We describe two patients with solid-pseudopapillary tumour of the pancreas. A smaller, localised tumour in an unusually young white man was surgically excised with no evidence of recurrence after 2 years. The other case also had an uncommon presentation, with an aggressive course resulting in vascular encasement of the superior mesenteric bundle and aorta, and local involvement of the mesenteric lymph nodes. A literature review was carried out, and the main clinico-pathological features and strategies of treatment of solid-pseudopapillary tumour of the pancreas are presented. Pathological, genetic and molecular features distinguish solid-pseudopapillary tumours from pancreatic ductal adenocarcinoma. Furthermore, neuroendocrine differentiation can be found focally in occasional cases of solid-pseudopapillary tumour. patients with localised disease are usually cured by surgery. Prolonged survival can be seen in the presence of distant metastasis, if such lesions are resected surgically. Chemotherapy and radiation therapy are used in rare cases when resection is not possible. No current chemotherapy regimens are considered standard in the treatment of this tumour. A rational chemotherapy protocol for such a rare tumour needs to consider its origin and clinical behaviour. However, the indolent clinical progression of solid-pseudopapillary tumours is similar to that of pancreatic neuroendocrine tumour.
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8/14. Papillary thyroid carcinoma with exuberant nodular fasciitis-like stroma: treatment outcome and prognosis.

    Papillary thyroid carcinoma with nodular fasciitis-like stroma (PTC-NFS) is one of the extremely rare variants of papillary thyroid carcinoma. To date, the majority of reported cases have been published in the surgical pathology and cytopathology literature, addressing the diagnostic difficulties posed by the condition's extensive, reactive stromal proliferation. Because of the rarity of PTC-NFS among papillary thyroid carcinoma variants, it has been unexplored from a clinical viewpoint. A medline search on the clinical course, role of radioiodine, treatment outcome and long term follow up of this disease yielded no result.We report the clinicoradiologic and histopathologic profile, together with post-treatment long term follow up, in a 35-year-old woman harbouring this rare entity. To the best of our knowledge, this is the first report of a five-year follow up of this rare variant of PTC following total thyroidectomy and radioiodine treatment. Our follow-up findings reiterate the disease's favourable clinical course when managed in the same manner as a classical, differentiated papillary carcinoma of the thyroid, akin to that predicted by the pathologists, and emphasize the importance of differentiating PTC-NFS as a separate entity from the papillary carcinoma variants with aggressive histology. Given the rarity of this condition, the experience gained from the present case is a useful addition to the current knowledge on disease prognostication and management.A systematic review of the existing literature on PTC-NFS, including the case reported in the present paper, is also carried out, aiming to explore the patient characteristics and clinical behaviour pattern of this rare entity and to make appropriate recommendations on management strategy. The age of presentation ranges from 20 to 82 years, with a mean of 44.5 years. female preponderance was observed, with a female to male ratio of 3ratio1. No racial predilection was observed. Tumour size varied from 2 to 9 cm along its greatest diameter (mean = 4.3 cm). Metastasis to lymph nodes at presentation occurred in 25 per cent of cases. Metastasis to surrounding structures (e.g. parathyroid and skeletal muscle) was observed in 12.5 per cent. There have been no reports of pulmonary or skeletal metastasis at presentation.
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9/14. Solid pseudopapillary tumour of the pancreas: diverse presentation, outcome and histology.

    CONTEXT: Solid pseudopapillary tumour of the pancreas is an uncommon tumour, which predominantly occurs in young females and is of unknown origin. CASE REPORT: We describe five cases with diverse clinical and/or histological features, including one unusually aggressive case resulting in early death. CONCLUSION: There is great variability in the presentation and clinical course of these tumours with further research needed to define their histogenesis and biological behaviour.
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10/14. Papillary thyroid cancer with unusually aggressive behaviour. Case report.

    A 22-year-old woman underwent thyroid lobectomy for papillary carcinoma (follicular variant). Local recurrences 4 and 7 months later, with vascular invasion and extrathyroid spread, required extensive surgery and radiotherapy. Ipsilateral lobectomy with near-total or total contralateral lobectomy is advocated for treatment of papillary thyroid cancer.
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