Spindle cell oncocytoma of the neurohypophysis with metastasis to the sphenoparietal sinus and immunohistochemical negativity for S100 and epithelial membrane antigen (EMA).

We report the case of a 61-year-old male with spindle cell oncocytoma of the hypophysis. On presentation to the Department of Neurosurgery at the German Armed Forces Hospital of Ulm, the patient reported a history of several years of left sixth nerve palsy, right ptosis, increased sensitivity to light, and a bilateral retrobulbar pressure sensation. Pituitary function was normal. A chromophobe non-functioning pituitary adenoma was initially suspected. The diagnosis was established on the basis of examination at a histopathology reference laboratory using immunohistochemistry to identify cell surface markers. During two years of follow-up, there were two clinical recurrences requiring surgery. To our knowledge, this is the 35th documented case of spindle cell oncocytoma of the pituitary gland and the first that was immunohistochemically negative for epithelial membrane antigen (EMA) and S100; and the first that displayed haematogenous metastasis to the right sphenoparietal sinus. The three surgical procedures were associated with massive intraoperative bleeding and thus resulted in subtotal tumor resection. Following surgery for the recurrences, the patient underwent radiotherapy.

Feminizing adrenocortical oncocytoma presenting as precocious puberty: a case report and literature review.

OBJECTIVES: Feminizing adrenal tumors are rare in childhood. We present a case of a special category of adrenal tumor, an oncocytoma, causing isosexual peripheral precocity. CASE PRESENTATION: A 4-year old girl presented with breast development and menstrual bleeding over a period of 3-4 months. Her SMR staging was breast stage 4, pubic hair stage 3. Her bone age was advanced (6 year 10 months), stimulated LH 0.7 IU/L, estradiol 206 pmol/L and DHEAS >27.1 micromol/L. CT scan revealed a right adrenal mass with features of atypical adrenal adenoma. Laparoscopic adrenalectomy was done and histopathology revealed oncocytoma. Lin-Weiss-Bisceglia criteria classified it as likely benign, borne out till a 2 year follow up. CONCLUSIONS: Adrenal oncocytoma can be a cause of isosexual peripheral precocity in a young girl. Recognition and correct classification of this histological variant, which is more often benign, is important for prognostication and choice of therapy after surgery.

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.

Growth and renal function dynamics of renal oncocytomas in patients on active surveillance.

OBJECTIVES: To study the natural history of renal oncocytomas and address indications for intervention by determining how growth is associated with renal function over time, the reasons for surgery and ablation, and disease-specific survival. PATIENTS AND METHODS: The study was conducted in a retrospective cohort of consecutive patients with renal oncocytoma on active surveillance reviewed at the Specialist Centre for Kidney Cancer at the Royal Free London NHS Foundation Trust (2012 to 2019). Comparison between groups was performed using Mann-Whitney U-tests and chi-squared tests. A mixed-effects model with a random intercept for patient was used to study the longitudinal association between tumour size and estimated glomerular filtration rate (eGFR). RESULTS: Longitudinal data from 98 patients with 101 lesions were analysed. Most patients were men (68.3%) and the median (interquartile range [IQR]) age was 69 (13) years. The median (IQR) follow-up was 29 (26) months. Most lesions were small renal masses, and 24% measured over 4 cm. Over half (64.4%) grew at a median (IQR) rate of 2 (4) mm per year. No association was observed between tumour size and eGFR over time (P = 0.871). Nine lesions (8.9%) were subsequently treated. Two deaths were reported, neither were related to the diagnosis of renal oncocytoma. CONCLUSION: Natural history data from the largest active surveillance cohort of renal oncocytomas to date show that renal function does not seem to be negatively impacted by growing oncocytomas, and confirms clinical outcomes are excellent after a median follow-up of over 2 years. Active surveillance should be considered the 'gold standard' management of renal oncocytomas up to 7cm.

Four synchronous primary tumors in a male patient.

Multiple primary malignancies are defined as two or more primary malignant tumors diagnosed in one individual; they are further classified to synchronous or metachronous based on the period between each cancer diagnosis and the other. The diagnosis of four synchronous cancers is exceedingly rare. We report a case of a 72-year-old man, diagnosed with synchronous quadruple cancers, Hurthle cell carcinoma and papillary carcinoma of the thyroid, as well as squamous cell carcinoma and carcinoid tumor of the lung.

Synchronous eyelid oncocytoma and conjunctival intraepithelial neoplasia

Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.

Hurthle cell carcinoma in childhood: A retrospective analysis of five cases and review of pediatric literature.

BACKGROUND: the available studies on Hurthle cell carcinoma (HCC) in pediatric age are scarce and based on isolated case reports. Aims of the present study were to review the available pediatric literature on HCC (2000-2019), to describe the cohort of children with this cancer histotype, and to estimate its relative prevalence in pediatric age. PROCEDURE: We retrospectively reconstructed an HCC course in five patients < 19 years who were identified in our departments during the period 2000-2019, and we reviewed the available pediatric studies on this differentiated thyroid cancer (DTC) variant. RESULTS: HCC occurred with a relative prevalence of 5.8% at a median chronological age of 12.5 years. None of HCC patients exhibited, at diagnosis, thyroid dysfunction, extensive lateral neck disease, or distant metastases, and all showed a persistent remission over time. Three patients showed, at diagnosis, antecedents of other diseases (Hashimoto's thyroiditis, neurofibromatosis type 1, and osteosarcoma). CONCLUSIONS: (1) In childhood, the relative prevalence of HCC among different thyroid cancer histotypes is 5.8%, that is close to the one previously reported both in the general population and in other less numerous children's cohorts; (2) HCC may develop even early, at the age of 7; (3) in childhood, HCC does not seem to have a more aggressive behavior when compared with other DTC histotypes; (4) antecedents of other diseases are not infrequent in the history of children with HCC.

Hürthle Cell Thyroid Adenoma Showing Avid Uptake on 18F-PSMA-1007 PET/CT.

We report the case of a 74-year-old man with suspected recurrent prostate carcinoma who underwent F-PSMA-1007 PET/CT for restaging the disease. PET/CT imaging presented an incidentaloma with intense F-PSMA-1007 uptake in the right thyroid lobe. Biopsy and genomic sequencing test confirmed the diagnosis of Hürthle cell thyroid adenoma. Therefore, it is important to raise awareness of this benign lesion in the differential diagnosis of incidental thyroid nodules with high F-PSMA-1007 activity.

Tumors of the Neurohypophysis: One Unit's Experience and Literature Review.

OBJECTIVE: To evaluate and understand the clinical behavior and radiologic correlates of tumors originating from the posterior pituitary gland. To review the management strategy for these rare tumors and add to the limited existing literature. METHODS: Retrospective review of 8 cases (5 pituicytomas, 2 spindle cell oncocytomas, and 1 granular cell tumor) managed at our institution between 2004 and 2019. The patients' clinical course, histologic features, and radiologic findings were reviewed. Their management and long-term follow-up is presented and compared with the literature. RESULTS: Long-term follow-up ranged from 1 to 9 years. There was 1 recurrence in a patient with spindle cell oncocytoma, and this was treated with radiotherapy. The endoscopically managed cases resulted in complete tumor excision with no recurrence. CONCLUSIONS: Epidemiologic data on primary tumors of the neurohypophysis is limited because of the rarity of these tumors. This study adds to the literature that these tumors behave as World Health Organization grade I tumors, although close follow-up is recommended as a few cases have shown recurrence. The endoscopic approach resulted in better gross total tumor resection rate in this series.

Unusual cause of airway obstruction: subglottic oncocytic cyst.

Oncocytic cysts are rare benign cystic lesions, which are usually seen in the supraglottic region. They usually present with symptoms of hoarse voice or the sensation of a lump in the throat. Management is with complete excision and follow-up to monitor for recurrence. In this unusual case, an elderly woman presenting with shortness of breath following recent treatment for pneumonia developed rapidly worsening stridor during admission. Flexible nasal endoscopy revealed a large pedunculated cystic lesion arising from the subglottic laryngeal mucosa. A tracheostomy was performed as an emergency and the lesion was then excised. Histology confirmed the diagnosis of a benign oncocytic cyst. Although oncocytic cysts are a recognised benign pathology of the supraglottic larynx, they should remain in the differential diagnosis for the subglottic larynx. Management is based on cases of supraglottic pathology, and we advise careful monitoring for this previously unreported pathology.

Unusual Presentation of Hurthle Cell Carcinoma With TENIS Syndrome as Left Atrial Thrombus on 18F-FDG PET/CT.

Differentiated thyroid cancer (DTC) with thyroglobulin elevation and negative I scintigraphy (TENIS) syndrome is a diagnostic and therapeutic dilemma. Treatment of differentiated thyroid cancer has an excellent outcome, but treatment options in TENIS are limited with the exception of surgically amenable disease. We present a case of TENIS syndrome with incidental detection of tumor thrombus in right pulmonary vein extending to the left atrium on F-FDG PET/CT and subsequently complicating as an occipital bleed. The timely detection of the thrombus facilitated early initiation of anticoagulant therapy. However, the intracranial bleed precluded the continuation of anticoagulant therapy.

Hürthle Cell Carcinoma in a Lingual Thyroid.

OBJECTIVE: To present a case of lingual thyroid Hürthle cell carcinoma (HCC). CLINICAL PRESENTATION AND INTERVENTION: A 37-year-old female presented with dysphagia and recurrent haemorrhage. Histopathology was suggestive of HCC; the tumour was excised by the trans-glossal approach which provided adequate exposure and helped avert external scarring or mandibular osteotomy. Histopathology showed a tumour-positive right lateral resection margin. This prompted referral to nuclear medicine for radio-iodine ablation. CONCLUSION: Lingual thyroid cases should be followed up closely and fine-needle aspiration biopsy should be considered when in doubt.

Complementary role of parathormone washout test to 99mTc-MIBI parathyroid scintigraphy and histopathologic analysis of cell types in parathyroid adenomas. / Gammagrafía paratiroidea con 99mTc-MIBI: valor complementario de la paratohormona en el aspirado y análisis histopatológico de los tipos de células en los adenomas paratiroideos.

OBJECTIVE: Parathyroid scintigraphy (PS) can be negative or equivocal (N/E) in a considerable number of cases with highly suspicious clinical findings and biochemical results for parathyroid adenoma (PA). The aims of this study were to investigate the complementary role of parathormone washout test (PWT) to PS in patients with primary hyperparathyroidism (PHPT) and evaluate histopathologic aspects of PAs in comparison with PS results. MATERIAL AND METHODS: Thirty-eight patients with PHPT referred for PS were included in the study. Seventeen patients had both scintigraphic and ultrasonographic findings concordant with PA (Group A). Twenty-one patients having N/E PS, but suspected lesions for PA on ultrasonography (US) formed Group B. PWT was performed for all patients and they underwent the surgical intervention. An adenoma was removed in all patients and the histopathologic cell characteristics were established. RESULTS: The tumor size on US was larger in those patients whose adenomas were seen on the PS (P<.001). The percentages of chief (or principal), oxyphilic and clear cells in PAs were not statistically different between the groups. Serum parathormone level and PWT were not statistically significant between Group A and Group B (P=.095 and P=.04, respectively). CONCLUSION: Although there is not a definitive threshold value, the sensitivity of PS increases with lesion size. While chief cell and oxyphilic cell content of PAs tend to deplete in N/E PS, clear cell rate increases substantially. Combining PS with both US and PWT increases the sensitivity of detection and localization of PAs.

Nasal oncocytoma causing unilateral epiphora in an elderly patient: the vital role of nasendoscopy.

Oncocytomas are rare glandular tumours, typically found in salivary glands and kidneys. A 76-year-old man presented to an ophthalmology department after 3 months of persistent right-sided epiphora. Lacrimal syringing and a fluorescein dye test demonstrated an obstruction at the level of the nasolacrimal duct (NLD). Owing to the patient's history of dacryocystitis, endoscopic dacryocystorhinostomy was organised. When in theatre however, nasendoscopy identified a polypoid mass at the lateral aspect of the inferior turbinate, obstructing the right NLD opening. Histopathology confirmed an oncocytoma. There was no local destruction or distant metastases. The mass was resected, a Monoka stent inserted and symptomatic relief achieved. In this case, the cause of epiphora was initially missed because no direct nasal examination was performed. It is vital that patients with unilateral epiphora undergo an endoscopic nasal examination before arranging treatment. This could prevent delayed diagnoses, ensure prompt and appropriate management, and reduce morbidity and mortality.

Spindle cell oncocytoma: Report of two cases with massive bleeding and review of the literature.

Spindle cell oncocytoma (SCO) is a rare pituitary tumor, classified as a WHO grade I neoplasm. Due to its rarity, SCO is often preoperatively misdiagnosed as a pituitary macroadenoma. In the present study we report two recent cases of SCO, a 61-year-old male and a 65-year-old female presenting at Treviso General Hospital between March 2014 and April 2015. Tumor resection was achieved by endoscopic transsphenoidal approach but massive hemorrhagic events hampered surgery, endangering the patient's life in both cases. Both tumors featured fascicles of spindle cells with eosiniphilic cytoplasm expressing vimentin, S-100 and thyroid transcription factor-1 (TTF-1). The diagnosis of SCO was confirmed by second opinion in both cases. Extensive review of available literature, about 30 cases from 2002 to 2015, provided valuable clinical data for preoperative diagnosis and surgical removal of SCO tumors.