Endokrinologische Pathologie

Clinical PresentationA 53-year-old female presented with an abnormal swelling in the neck and a persistent cough. She complained of lethargy and weight gain over the previous few years. Whilst being investigated she died of unrelated cardiovascular disease several months later.PathologyThe specimen, removed at post-mortem, includes the base of the tongue, larynx and trachea. It has been cut in the coronal plane to allow a view of the internal laryngeal and tracheal anatomy. The thyroid gland is grossly enlarged particularly the right lobe, which extends superiorly and inferiorly, well beyond its normal margins when viewed from the anterior aspect. The cut posterior surfaces display many hyper- and hypopigmented nodules as well as cystic areas in both lobes. The tongue base, larynx and trachea appear relatively normal.Further informationNodular goitre is most often detected simply as a mass or swelling in the neck but depending on size and location of growth may produce pressure symptoms on the trachea and the oesophagus. There may be difficulty in breathing, dysphagia, cough, and hoarseness. Paralysis of the recurrent laryngeal nerve may occur by an expanding goitre, but this is rare. Symptoms suggesting obstruction of the trachea including cough, stridor and shortness of breath may occur. Occasionally tenderness and a sudden increase in goitre size arise due to cystic expansion or haemorrhage into a nodule[1].Causes of goitre include autoimmune disease (Hashimoto’s thyroiditis, Grave’s disease), the formation of one or more thyroid nodules and iodine deficiency. Goitre occurs when there is reduced thyroid hormone synthesis secondary to biosynthetic defects and/or iodine deficiency, leading to increased thyroid stimulating hormone (TSH). This stimulates thyroid growth as a compensatory mechanism to overcome the decreased hormone synthesis. Elevated TSH is also thought to contribute to an enlarged thyroid in the goitrous form of Hashimoto thyroiditis in combination with fibrosis secondary to the autoimmune process in this condition. In Grave’s disease, the goitre results mainly from stimulation by the TSH receptor antibody[1].Reference1. Hughes et al. (2012) Goitre: Causes, investigation and management. Aust Family Physician, 41, 572-576.

Clinical PresentationA 60-year-old female presented with an abnormal swelling in the neck, persistent cough and difficulty swallowing. She gained weight over the previous few years. She died of unrelated cardiovascular disease and the specimen was obtained at post-mortem.PathologyThe specimen, removed at post-mortem, includes the larynx, trachea and large multilobular thyroid gland. The thyroid gland is grossly enlarged particularly the right lobe, which has two large lobes extending superiorly and inferiorly for a range of 7-8mm, well beyond its normal margins when viewed on anterior aspect. Posteriorly, the oesophagus has been opened to expose the posterior wall of the trachea. The right lobe presents as larger than from the anterior perspective, and the abnormal growth appears to be mainly the inferior pole of the right lobe. The surfaces do not display major pigmentary changes. Prominent veins are visible on the surface of the right lobe.Further informationGoitre is most often detected simply as a mass or swelling in the neck but depending on the size and location of growth it may produce pressure symptoms on the trachea and the oesophagus. There may be difficulty in breathing, dysphagia, cough, and hoarseness. Paralysis of the recurrent laryngeal nerve may occur by an expanding goitre, but this is rare. Symptoms suggesting obstruction of the trachea including cough, stridor and shortness of breath may occur. Occasionally tenderness and a sudden increase in goitre size arise due to cystic expansion and haemorrhage into a nodule [1].Causes of goitre include autoimmune disease (Hashimoto’s thyroiditis, Grave’s disease), the formation of one or more thyroid nodules and iodine deficiency. Goitre occurs when there is reduced thyroid hormone synthesis secondary to biosynthetic defects and/or iodine deficiency, leading to increased thyroid stimulating hormone (TSH). This stimulates thyroid growth as a compensatory mechanism to overcome the decreased hormone synthesis. Elevated TSH is also thought to contribute to an enlarged thyroid in the goitrous form of Hashimoto thyroiditis in combination with fibrosis secondary to the autoimmune process in this condition. In Grave’s disease, the goitre results mainly from stimulation by the TSH receptor antibody[2].Reference1. Hughes et al. (2012) Goitre: Causes, investigation and management2. Aust Family Physician, 41, 572-576.

Clinical HistoryThis was a case of a 75-year-old female who presented with symptoms of recurrent disease and confirmed to have chemo-resistant multiple retroperitoneal lymph node metastases five years after the initial therapy for stage IIIc serous adenocarcinoma of the ovary. Positron emission tomography/computed tomography (PET/CT) revealed the involvement of para-aortic nodes and pelvic nodes. She died of liver complications before therapeutic options, such as radical lymphadenectomy, could be considered.PathologyThe specimen consists of the abdominal aorta and common iliac arteries surrounded by large numbers of extremely enlarged iliac nodes para-aortic lymph nodes. Histopathological examination revealed metastatic high-grade adenocarcinoma in some of the resected lymph nodes.Further informationOccasionally, lymph node metastases represent the only component at the time of recurrence of ovarian cancer. In this case, the metastatic nodes predicted by PET/CT completely corresponded to the actual metastatic nodes. Ultrasound (US) could equally have confirmed the presence of such large lymph nodes. PET/CT or US often fails to identify microscopic disease in histopathologically-proven positive nodes. Therefore, it is difficult to reliably exclude lymph node metastases during surveillance following initial surgery for ovarian cancer.In the context of a recurrent ovarian disease, systematic aortic and pelvic node dissection would be considered appropriate in younger women with no other evidence of metastatic disease. This is unlikely to be curative but may produce palliation with symptom control and allow for the trial of novel therapies if available.More commonly, sampling of pelvic and aortic lymph nodes is part of the formal surgical staging of epithelial ovarian cancer at the time of initial surgical treatment. In addition, in women presenting initially with advanced stage ovarian cancer, systematic debulking of enlarged retroperitoneal lymph nodes will be considered if this leads to complete debulking of the tumour.

Clinical HistoryA 68-year old woman presented with a small hard lump in the thyroid. During the following six weeks, there was progressive rapid enlargement of the mass leading to laryngeal stridor and oesophageal obstruction, which were the terminal features. No lymph nodes were enlarged and there was no splenomegaly.PathologyThe larynx, thyroid, upper trachea and oesophagus are included in the specimen. The enlarged left lobe and to a lesser degree, the right lobe of the thyroid, are replaced by homogeneous pale tumour tissue. Stretched over the lateral margin of the left lobe is the common carotid artery. Note on the internal aspect how the larynx is compressed and the oesophagus virtually disappears into the bulk of the tumour. The histological appearance of the tumour was consistent with lymphoblastic lymphoma of the thyroid. The rarity of this tumour makes it necessary to exclude the diagnosis of anaplastic carcinoma of the thyroid, and secondary spread from lymphoma elsewhere.Further InformationPrimary lymphoma of the thyroid is rare but should always be considered as a differential diagnosis of any thyroid mass or nodule. Primary thyroid lymphomas are rare, and are usually of the non-Hodgkin type. Lymphoblastic lymphoma is an aggressive form of non-Hodgkin’s lymphoma (NHL), usually seen in children. Currently, the only known risk factor for developing primary thyroid lymphoma is chronic autoimmune (Hashimoto’s) thyroiditis, with a 50% occurrence rate.Thyroid lymphoma should be considered in any individual presenting with a rapidly enlarging ‘goitre’, as over 90% of individuals with this disease present this way. As the goitre enlarges, there can be compression of nearby structures such as the trachea, oesophagus and neck vessels (the common carotid in this case). The resulting symptoms include stridor or hoarseness, dysphagia and neck pain. Other systemic symptoms of lymphoma include so-called B-symptoms, i.e. night sweats, fevers and loss of weight.Diagnosis can be achieved by an ultrasound scan followed by fine needle aspiration or excisional biopsy. As thyroid lymphomas cannot be excluded from Hashimoto’s or other carcinoma, cytology and immunohistochemical stain of the biopsied tissue is required to confirm the diagnosis.