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      A case of thyroid fibromatosis, a rare lesion of this gland

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          Summary

          Thyroid fibromatosis is a very rare lesion; to our knowledge, there are only four cases reported in the medical literature. Herein, we report the clinical case of a woman with thyroid fibromatosis with a long follow-up (11 years). A 63-year-old female patient, with an increasing multinodular goitre without compressive symptoms, was admitted to total thyroidectomy. The histology revealed a spindle-cell proliferation with fibroblastic characteristics with no atypia and thin capillary vessels. Immunohistochemistry was positive for beta-catenin, focally to desmin and alfa-actin and negative for cytokeratins and CD34. Thyroid cells did not display any features of papillary thyroid cancer. These characteristics were compatible with thyroid fibromatosis. For the past 11 years, the patient has been periodically followed up with neck CTs and she has not shown any signs of recurrence. Thyroid fibromatosis has been associated with invasion of surrounding structures in previous reported cases. However, this aggressive behaviour was not observed in our patient. The most challenging differential diagnosis is with papillary thyroid cancer with fibromatosis-like stroma, in which the malignant component is usually peripheral. Therefore, in these cases, it is mandatory to perform an extensive examination of the resected sample.

          Learning points:

          • Fibromatosis is a mesenchymal lesion that consists of an infiltrative proliferation of fibroblasts without atypia.

          • Thyroid fibromatosis is a rare entity in this gland. In previously reported cases, it has been associated with an invasive behaviour but this was not the case in our patient.

          • When spindle-cell proliferation with fibroblastic/myofibroblastic characteristics is detected on thyroid histology, it is mandatory to exclude a papillary thyroid carcinoma with fibromatosis-like stroma.

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          Most cited references9

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          Desmoid tumors in patients with familial adenomatous polyposis.

          Sporadic desmoid tumors occur mainly in the abdominal wall and in extraabdominal sites. Desmoid tumors in patients with familial adenomatous polyposis (FAP) usually occur in the abdominal wall and in the bowel mesentery. Surgical resection of desmoids in patients with FAP has been controversial. A retrospective review of patients with FAP and desmoid tumors treated from 1950 to 1991 was performed. Patients were evaluated for gender, age, site of desmoid tumors, treatment, recurrence, and survival. Twenty-one of 24 patients underwent 60 surgical procedures related to the desmoid tumors. Seven of nine patients who underwent potentially curative surgery had recurrences; three were reresected. Major morbidity after palliative or curative surgery was 47%. Five patients were alive with no evidence of disease at a median of 198 months, 10 patients were alive with disease at a median of 102 months, and 5 patients died with disease at a median of 31 months after diagnosis. Desmoid tumors are common in patients with FAP. Unresectability and recurrence are more common than cure. Palliative and curative resections have a high morbidity. Surgery should be reserved for those patients with symptomatic mesenteric desmoids.
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            Papillary carcinoma of thyroid with exuberant nodular fasciitis-like stroma. Report of three cases.

            Three examples of an unusual morphologic variant of papillary thyroid carcinoma (PTC) are reported. The presence of a prominent stromal component resulted in low-power microscopic appearances resembling fibroadenoma, phyllodes tumor, or fibrocystic disease of the breast in two cases. The carcinomatous component grew in the form of anastomosing narrow tubules, clustered glands, solid sheets with or without squamous differentiation, and/or papillae, and exhibited the typical nuclear features of PTC. The abundant stroma had a nodular fasciitis-like quality and was composed of short fascicles of spindle cells separated by varying amounts of mucoid matrix, collagen, and extravasated red blood cells; this was interpreted as an exuberant mesenchymal reaction to the carcinoma. The importance of recognizing this variant of PTC is that, when one encounters a fibroproliferative lesion of the thyroid, a diligent search should be made for papillary carcinoma. This variant also must be distinguished from the vastly more aggressive papillary carcinomas with anaplastic transformation and the so-called carcinosarcomas.
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              Aggressive fibromatosis of the head and neck (desmoid tumours).

              Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                11 October 2016
                2016
                : 16-0019
                Affiliations
                [1 ]Serviço de Endocrinologia
                [2 ]Unidade de Investigação em Patobiologia Molecular (UIPM) , Instituto Português de Oncologia de Lisboa, Francisco Gentil, Lisbon, Portugal
                [3 ]NOVA Medical School/Faculdade de Ciências Médicas , Universidade Nova de Lisboa, Lisbon, Portugal
                [4 ]Serviço de Anatomia Patológica , Instituto Português de Oncologia de Lisboa, Francisco Gentil, Lisbon, Portugal
                Author notes
                Correspondence should be addressed to Joana Simões-Pereira; Email: joanasimoespereira@ 123456gmail.com
                Article
                EDM160019
                10.1530/EDM-16-0019
                5093403
                27855230
                f3456611-09d9-4a81-a1fd-39c93cae6087
                This is an Open Access article distributed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 9 August 2016
                : 16 September 2016
                Categories
                A Case of Thyroid Fibromatosis, a rare lesion of this gland

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