Masson's papillary endothelial hyperplasia mimicking a poorly differentiated thyroid carcinoma: a case report

Autor: Annette Winkler, Peter Diem, Bruno Mueller
Rok vydání: 2001
Předmět:
Zdroj: European Journal of Endocrinology. :667-668
ISSN: 1479-683X
0804-4643
DOI: 10.1530/eje.0.1450667
Popis: Intravascular papillary endothelial hyperplasia (PEH,Masson’s tumour) is a benign lesion of vascular originthat is caused by an excessive and atypical proliferationof endothelial cells in normal blood vessels or vascularmalformations. PEH may mimic angiosarcoma or othertumours (1). On only very rare occasions does PEHoccur extravascularly as a result of the organization ofa haematoma (2), although a history of trauma isseldom elicited. There are only two reported cases inthe literature of PEH of the thyroid, one occurredsubsequent to multiple fine-needle aspiration biopsies,the second occurred in an organizing haematomawithin a hyperplastic follicular nodule (3, 4). Wepresent here what we believe to be the first report of apatient with PEH of the thyroid that was mistaken for apoorly differentiated thyroid carcinoma on the originalhistology.A 45-year-old woman presented in 1988 with a non-toxic multinodular goitre. Her medical history revealedno significant, pre-existing disease and her sister alsosuffered from goitre. Because of neck discomfort, thepatient underwent a subtotal thyroidectomy. Histologi-cal examination revealed foci with hyperplasia of theepithelial cells, colloid-rich adenomatoid nodules withhaemorrhagic and cystic degeneration. Furthermore, anarea with round to spindle-shaped cells with a highmitotic activity and infiltrative expansion exceeding thethyroid capsule was detected. Results from an immuno-histochemical analysis of this area were negative forsynaptophysin and calcitonin. The histopathologicalfeatures were interpreted byan approved pathologist as apoorly differentiated thyroid carcinoma. Complete thy-roidectomy was therefore carried out; total ablation withiodine-131 was not performed. Suppressive levothyrox-ine therapy was initiated. One year postoperatively, athyroid radionuclide scan in combination with anultrasound revealed an isolated 50 mm recurrent masson the left side; lymph node metastases were notdetected. Results of an iodine-131 total body scan werenegative. Serum thyroglobulin concentrations wereelevated (122 ng/ml; reference range ,25) but calci-toninvalues were within the normal range. Apart from afollow-up including serum thyroglobulin and calcitoninmeasurements no further steps were taken.In 1996 serum thyroglobulin concentrationsincreased rapidly (885 ng/ml). An ultrasound of thethyroidshoweda57 mmnodulewithnon-homogeneousechogenicity. Thyroid scintigraphy using 99Tc-pertech-netate and 99mTc-MIBI showed a positive traceraccumulation within this mass. Based on thesefindings, the patient was referred to our endocrinedivision, and the tumour tissue in the neck wasremoved. Histopathological evaluation revealed typicalfindings of a simple nodular goitre; there were no signsof malignancy. Because of the very unusual course ofthis tumour, the original specimens which had beenconserved were histologically and immunohistochemi-cally reanalysed. The spindle-shaped cells were foundmainly to be endothelial cells with papillary prolifera-tion within dilated venules. The additional immuno-histochemical investigations showed positive stainingto vimentin, to CD34 and to factor VIII-related antigenwithin the endothelial cells. Thus, histopathologicalexamination revealed a pure form of PEH; the originaldiagnosis of poorly differentiated thyroid carcinomawas dismissed.Intrathyroidal PEH is a very rare entity, but may havebeen under-reported in the literature (5). Because of itsclinical course and histological findings, PEH may bemistaken for a malignant tumour, as is illustrated byour case. Misdiagnosis may lead to consequences suchas inappropriate radical surgery. PEH can be cured bycomplete surgical resection. Our patient thereforeunderwent two unnecessary operations to remove therecurrent ‘malignant tumour’. It is also important todistinguish between these two lesions to avoid in-appropriate medical treatment such as chemotherapy,total ablation with iodine-131 or levothyroxine-suppressive treatment. The latter has potentiallyserious side-effects, as has been described recently (6).The psychological aspect of our patient living with thediagnosis of malignant tumour must also be con-sidered. Physicians caring for patients with thyroiddisorders and pathologists should therefore be aware ofPEH and suspect its presence.
Databáze: OpenAIRE
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