Graves disease and metastatic hormonal-active Hürthle cell thyroid cancer: A case report.

RATIONALE: A hormone-active metastatic Hürthle cell thyroid carcinoma (HCTC) and Graves disease (GD) present a therapeutic challenge and is rarely reported. PATIENT CONCERNS: We present a 64-year-old male patient, who had dyspnea and left hip pain lasting 4 months. He had clinical signs of hyperthyroidism and a tumor measuring 9 cm in diameter of the left thyroid lobe, metastatic neck lymph node and metastases in the lungs, mediastinum, and bones. DIAGNOSIS: Laboratory findings confirmed hyperthyroidism and GD. Fine-needle aspiration biopsy and cytological investigation revealed metastases of HCTC in the skull and in the 8th right rib. A CT examination showed a thyroid tumor, metastatic neck lymph node, metastases in the lungs, mediastinum and in the 8th right rib measuring 20 × 5.6 × 4.5 cm, in the left acetabulum measuring 9 × 9 × 3 cm and parietooccipitally in the skull measuring 5 × 4 × 2 cm. Histology after total thyroidectomy and resection of the 8th right rib confirmed metastatic HCTC. INTERVENTIONS: The region of the left hip had been irradiated with concomitant doxorubicin 20 mg once weekly. When hyperthyroidism was controlled with thiamazole, a total thyroidectomy was performed. Persistent T3 hyperthyroidism, most likely caused by TSH-R-stimulated T3 production in large metastasis in the 8th right rib, was eliminated by rib resection. Thereafter, the patient was treated with 3 radioactive iodine-131 (RAI) therapies (cumulative dose of 515 mCi). Unfortunately, the tumor rapidly progressed after treatment with RAI and progressed 10 months after therapy with sorafenib. OUTCOMES: Despite treatment, the disease rapidly progressed and patient died due to distant metastases. He survived for 28 months from diagnosis. LESSONS: Simultaneous hormone-active HCTC and GD is extremely rare and prognosis is dismal. Concomitant external beam radiotherapy and doxorubicin chemotherapy, followed by RAI therapy, prevented the growth of a large metastasis in the left hip in our patient. However, a large metastasis in the 8th right rib presented an unresolved problem. Treatment with rib resection and RAI did not prevent tumor recurrence. External beam radiotherapy and sorafenib treatment failed to prevent tumor growth.

[The diagnostic and therapeutic challenges of bilateral renal oncocytosis : Illustrative case presentations and a review of the literature]. / Die bilaterale renale Onkozytose als diagnostische und therapeutische Herausforderung : Falldarstellung und Literaturreview.

Bilateral oncocytosis along with multiple tumours in both kidneys represents a very rare pathology that is accompanied by diagnostic and therapeutic challenges. We report the case of a 60-year old male patient who underwent computer tomography with incidental detection of multiple bilateral and contrast enhancing renal tumours of different size. Subsequently the patient underwent nephron-sparing tumor resection, first on the right side and 4 weeks later on the left side. The histology of all removed tumors showed evidence of pure oncocytoma. There were no postoperative complications and renal function reached a stable state within 6 months follow-up. The major challenge regarding diagnostic process and therapy of this pathology is to distinguish benign oncocytoma from chromophobe renal cell carcinoma and hybrid tumours, which can all be associated with renal oncocytosis. Because of limitations concerning imaging processes and biopsy, all patients should undergo nephron-sparing surgery as far as possible. On the other hand alternative therapies should - regarding to therapy-associated morbidity and the basically benign prognosis of oncocytoma - be well discussed to obtain informed consent. In this case report different therapy options and the international literature concerning renal oncocytosis will be discussed.

Parathyroid carcinoma and oxyphil parathyroid adenoma: an uncommon case of misinterpretation in clinical practice.

A 46 year-old male presented with persistently high level of serum parathyroid hormone (PTH), despite successful resection of an oxyphilic cell parathyroid adenoma of the left lower gland. Renal function and serum calcium were normal, leading to vitamin D deficiency being considered. Tc99m-sestamibi parathyroid scintigraphy showed no capitation, but a cervical ultrasound demonstrated an increase in the lower parathyroids. Surgery confirmed that the right gland was normal but the left corresponded to parathyroid carcinoma. The patient developed severe hypocalcemia, with PTH values being consistent with hypoparathyroidism for a few months. However, a progressive increase in calcium and PTH serum levels indicated recurrence of disease. Tc99m-sestamibi scintigraphy demonstrated hyperfixation in topography of the left inferior parathyroid and the patient was subjected to a third and more extensive surgery, with removal of lymph nodes and adjacent thyroid tissue. Serum calcium and PTH remained elevated, requiring loop diuretics and intravenous bisphosphonates to control hypercalcemia. Cervical radiotherapy was implemented as adjuvant therapy. After two months the patient complained of dyspnea, and a CT scan of the chest demonstrated areas of parenchymal condensation, suggestive of actinic pneumonitis. At the 2-year follow-up no major issues were evident.

Masa ocupante renal sólida de hasta 4 cm: análisis de la metodología diagnóstica, estadificación TNM y tratamiento quirúrgico / Solid renal mass up to 4 cm. Analysis of the diagnostic procedures, TNM staging and surgical treatment

Objetivos: Analizar retrospectivamente tumores renales operados con un tamaño máximo de 4 cm (T1a) para determinar si la metodología diagnóstica fue adecuada, el valor de la estadificación TNM de la UICC 2002 sobre el pronóstico y evaluar el tratamiento quirúrgico de elección. Material y método: Se estudiaron 78 unidades renales de 74 pacientes operados (4 casos bilaterales sincrónicos) desde 1984 a 2005 en el Servicio de Urología del Hospital Alemán. Se analizó la distribución etaria, sexo, forma de presentación (incidental y sintomático), metodología diagnóstica, variables de laboratorio, tratamiento quirúrgico (cirugía parcial o radical), histopatología, grado de malignidad según Fuhrman, tamaño tumoral, bilateralidad, multicentricidad estadificación TNM, evolución y supervivencia con la curva de Kaplan Meier. Resultados: Se estudiaron 78 unidades renales de un total de 192, 40,62%. La edad media fue 58,72 años. Sexo masculino 69% y femenino 31%. La forma de presentación fue 91,90% incidentales y sintomáticos el 8,10% (Hematuria en 5 y MTS pulmonar en 1). El diagnóstico fue por ecografía y tomografía computarizada en 63 casos, en 4 se agregó resonancia nuclear magnética (RNM) y en 7 ecografía y RNM. Arteriografía en 11 casos, 3 informaron tumor y 8 fueron normales. Biopsia en 5 casos, todas positivas para carcinoma células claras. El laboratorio sólo halló alterada la eritrosedimentación en 12 casos. El tratamiento quirúrgico fue cirugía radical en 35 unidades renales (44,87%) y conservadora en 43 unidades (55,13%). La histopatología fue carcinoma a células claras(CC) 79,48%, carcinoma papilar 1,28%, angiomiolipoma (AML) 8,97%, oncocitoma 7,69% y adenoma 2,56%. El grado de malignidad fue Fuhrman 1(76,19%), 2(20,63%) y 3(3,18%). Bilaterales 4 en 2 sendos CC, 1 CC y AML y 1 CC y adenoma. Tamaño tumoral medio 2,93 cm. Estadios T1a 96,82%, T3a 1,59% y T3aM+1,59%. Se obtuvieron datos de la evolución en 54 de 61 tumores malignos, de los cuales 50 casos se hallaban libres de enfermedad, 3 fallecidos por progresión a los 18, 33 y 82 meses, respectivamente, todos ellos tumores sintomáticos y 1 fallecido por otra causa. La supervivencia con un tiempo medio de seguimiento de 52,25 meses fue 94%. Conclusiones: 1) Con la ecografía y la TC se obtuvo un alto índice de definición diagnóstica para tumores renales sólidos. La biopsia en ciertos casos contribuyó a lograr una correcta táctica de tratamiento. 2) La cirugía conservadora fue el tratamiento de elección en tumores hasta en 4 cm correctamente seleccionados. Creemos que la estadificación TNM debería contemplar en este tamaño tumoral la forma de presentación para mejorar su valor en el pronóstico

Objectives: We perform a retrospective review of renal tumors operated with a maximum size of 4 cm to determine if the diagnostic methodology was adequate, the TMN staging prognostic accuracy (UICC 2002) and the goal surgical treatment. Material and method: Between 1984 to 2005, 78 renal units form 74 patients (4 bilateral synchronous) operated at the Service of Urology of the Hospital Alemán de Buenos Aires. Age distribution, sex, presentation form (incidental and symptomatic), diagnostic methodology, laboratory variables, surgical treatment (partial or radical surgery), histopathology, Fuhrman grade, tumor size, bilaterality, multicentricity, TNM staging, evolution and survival (Kaplan Meier) were analyzed. Results: 78 renal units from a total of 192, 40.62% were analyzed. The median age was 58.72 years. 69% were male and 31% female. The presentation form was 91.90 % incidental and symptomatic 8.10% (Hematuria in 5 and lung metastasis in 1). The diagnosis was performing with ultrasound and CT scan 63 cases, in 4 was also used resonance magnetic imaging (RMI) and in 7 ultrasound and RMI. Arteriography was used in 11 cases, 3 showed tumor and 8 were normal. Biopsy was performing in 5 cases, all positive for clear cells carcinoma. Globular sedimentation was the only one laboratory abnormality in 12 cases. Surgical treatment was radical surgery in 35 renal units (44.87%) and conservative surgery in 43 units (55.13%). Pathology clear cells carcinoma (CCC) 79.48%, papillary carcinoma 1.28%, angiomyolipoma (AML) 8.97%, oncocytoma 7.69% and adenoma 2.56%. The Fuhrman grade was 1 in 76.19%; 2 in 20.63% and 3 in 3.18%. Bilateral tumor were found in 4 cases 2 CCC, 1 CCC and AML and 1 CCC and adenoma. Tumor median size was 2.93 cm. Staging was T1a 96.82%, T3a 1.59% and T3aM+ 1.59%. Follow-up could be made in 54 of 61cases. At median follow-up of 52.25 months, 50 cases were disease free, 3 died by progression at 18, 33 and 82 months and all of them were symptomatic tumors, 1 died by a non related cause. Survival rate was 94%. Conclusions: 1)Ultrasound and CT scan obtained a highest diagnostic accuracy for solid renal mass. Biopsy in selective cases could contribute to achieve a correct treatment strategy. 2) Conservative surgery was the goal treatment in selected tumors up to 4 cm. and we believe that TNM staging should contemplate the presentation form to improve the prognostic value

Lack of impact of radioiodine therapy in tg-positive, diagnostic whole-body scan-negative patients with follicular cell-derived thyroid cancer.

Several reports have suggested a benefit from radioactive iodine (RAI) therapy in Tg-positive, whole-body scan-negative patients with follicular cell-derived thyroid cancer, who were said to have high rates of visualization of uptake in metastases after therapeutic doses of RAI. We sought to evaluate the rate of visualization of RAI uptake in these patients and determine the effect of such therapy on tumor progression and Tg levels. We studied 24 consecutive patients who had been treated with high-dose RAI, four of whom had no evidence of metastasis or persistent cancer. Our results showed that four patients had some uptake in posttherapy scans: in the neck, lung, and mediastinal metastases in one patient, in the thyroid remnant in two, and in a possible neck microrecurrence in one. In 13 patients with macrometastases-tumors 1 cm or greater-tumors progressed and serum Tg increased; five have died of thyroid cancer. The disease remained stable in the seven patients with micrometastases. We concluded that in high-risk patients with follicular cell-derived thyroid cancer with high Tg levels and negative diagnostic whole-body scans, only a small number showed meaningful uptake after high doses of RAI. Therefore, widespread use of empiric RAI therapy for such patients who have a large tumor burden should not be encouraged.

Hürthle cell carcinoma.

Patients with Hürthle cell carcinoma (HCC) of the thyroid often have aggressive tumors and generally have a worse prognosis than those with papillary or follicular thyroid carcinomas. A total thyroidectomy with ipsilateral central neck lymphadenectomy and a modified radical neck dissection, if central or lateral nodes are positive, are indicated for HCC. The completeness of this procedure should be assessed by radioiodine scan 3 to 4 months after surgery. Any thyroid remnant should be ablated with radiolabeled iodine 131 to eliminate all tissue at risk and to facilitate the use of serum thyroglobulin in surveillance for tumor recurrence. Fewer than 10% of these cancers take up radioiodine. Recurrent disease is treated surgically with good palliation and appreciable prolongation of life. Local excision and neck dissection for recurrent neck disease or pulmonary wedge resection for lung metastasis has been shown to be effective. All patients with HCC should be given thyroid hormone because most of these tumors have thyrotropin receptors. External beam radiation may be considered for patients with unresectable disease, but this is considered palliative.

Malignant oncocytoma of major salivary glands. Report of a post-irradiation case.

The fourth case of malignant oncocytoma arising in the submandibular gland is here reported. This tumor arose in a 48-year-old man after radiation exposure, a finding never described before for malignant oncocytoma. In addition, several regional metastatic lymph nodes were found. The diagnosis was confirmed by histochemical and ultrastructural findings. The tumor cells showed easily recognizable mucus production and, ultrastructurally, abundant mitochondria, intracytoplasmic lumina lined by microvilli and lipid droplets. These last features have only seldom been described in malignant oncocytoma. Furthermore, the neoplastic cells were alpha-1-antitrypsin positive and S100, thyroglobulin, carcinoembryonic antigen, and smooth muscle actin negative. A thorough review of the literature is also presented.