Treatment modalities and outcomes of granular cell tumors and spindle cell oncocytomas of the pituitary gland: an analysis of two national cancer databases.

BACKGROUND: Spindle cell oncocytomas (SCO) and granular cell tumors (GCT) are rare primary pituitary neoplasms; the optimal treatment paradigms for these lesions are unknown and largely unexplored. Thus, using national registries, we analyze the epidemiology, management patterns, and surgical outcomes of SCOs and GCTs. METHODS: The National Cancer Database (NCDB; years 2003-2017) and the Surveillance, Epidemiology, and End Results Program (SEER; years 2004-2018) were queried for patients with pituitary SCOs or GCTs. Incidence, extent of surgical resection, and rate of postoperative radiation use for subtotally resected lesions comprised the primary outcomes of interest. All-cause mortality was also analyzed via time-to-event Kaplan-Meier curves. RESULTS: SCOs and GCTs have an annual incidence of 0.017 and 0.023 per 1,000,000, respectively. They comprise 0.1% of the benign pituitary tumors registered in NCDB. A total of 112,241 benign pituitary tumors were identified in NCDB during the study period, of which 83 (0.07%) were SCOs and 59 (0.05%) were GCTs. Median age at diagnosis was 55 years, 44% were females, and median maximal tumor diameter at presentation was 2.1 cm. Gross total resection was achieved in 54% patients. Ten patients (7%) had postoperative radiation. Comparing patients with GCTs versus SCOs, the former were more likely to be younger at diagnosis (48.0 vs. 59.0, respectively; p < 0.01) and female (59% vs. 34%, p = 0.01). GCTs and SCOs did not differ in terms of size at diagnoses (median maximal diameter: 1.9 cm vs. 2.2 cm, respectively; p = 0.59) or gross total resection rates (62% vs. 49%, p = 0.32). After matching SCOs and GCTs with pituitary adenomas on age, sex, and tumor size, the former were less likely to undergo gross total resection (53% vs. 72%; p = 0.03). Patients with SCOs and GCTs had a shorter overall survival when compared to patients with pituitary adenomas (p < 0.01) and a higher rate of thirty-day mortality (3.1% vs 0.0%; p = 0.013). CONCLUSION: SCOs and GCTs are rare pituitary tumors, and their management entails particular challenges. Gross total resection is often not possible, and adjuvant radiation might be employed following subtotal resection.

Scars Run Deep: Problematic Morphology and Immunoprofile of Scars in Renal Oncocytomas.

In some instances, the central scar of renal oncocytoma can demonstrate entrapped cells with unusual morphology and aberrant immunoprofile creating potential diagnostic confusion. Herein, 100 renal oncocytomas containing scars with embedded epithelial cells were identified from 6 institutions, including nephrectomies (64% partial, 36% radical) of similar laterality (left = 51%) and sex distribution (male = 56%), with patient ages ranging from 38 to 86 years (mean = 64.3years) and tumor sizes ranging from 2 to 16 cm (mean = 5.3 cm). Immunohistochemistry was performed on all tumors for KRT7, KIT, vimentin, and CA9 with staining intensity and extensity separately analyzed. Of 4 architectural patterns of cells within the scar, 60% showed tubular pattern. Of 4 cytologies within the scar, flat/elongated (49%) and cuboidal cells (40%) predominated. Within the scar, 62% showed eosinophilic cytoplasm, with 38% showing both cleared and eosinophilic cytoplasm; notably, 79% showed higher grade nuclei than typical oncocytes. A subset of scar cells showed mucinous-like basophilic secretions (19%). Compared to background renal oncocytoma, tumor cells within the scar were more often positive for vimentin, KRT7, and CA9 and more frequently negativity for KIT. Specifically, of the notable "aberrant" immunoprofiles, 79% showed KRT7 positivity/KIT negativity/vimentin positive, 84% showed vimentin positivity/CA9 positivity, and 78% showed KIT negativity/vimentin positivity/CA9 positivity. While encountering scars within renal oncocytomas is not uncommon, what is not well appreciated is the unique morphology and immunohistochemistry of tumor cells within the scar. Comparing tumor morphology and immunoprofile of the scar to the background oncocytoma is helpful to avoid interpretative confusion.

Renal oncocytoma in a 13-year-old girl: A case report and literature review.

Renal oncocytoma is a benign renal neoplasm which has mostly been reported in adults. Occurrence in children is infrequent. To date, there are only six pediatric cases of renal oncocytoma reported previously. Herein, we report a 13-year-old girl presented with hematuria for a week. Abdominal computed tomography showed a well-defined heterogeneous solid mass with a stellate central scar in the left kidney. The patient underwent a nephron sparing surgery. Histopathological and immunohistochemical findings confirmed the diagnosis of renal oncocytoma. Though uncommon, renal oncocytoma should be considered as the differential diagnosis of renal tumor in children. In addition, intranuclear inclusions were firstly described in this pediatric patient with unclear significance, which need a large cohort to summarize and analyze.

Oncocytic adrenocortical neoplasm of borderline uncertain malignant potential diagnosed after robot-assisted adrenalectomy case report.

BACKGROUND: Adrenal incidentalomas are radiologically discovered tumors that represent a variety of pathologies, with the diagnosis clinched only on surgical pathology. These tumors may be clinically monitored, but triggers for surgery include size > 4 cm, concerning features on radiology, or hormonally functioning. Adrenal oncocytic neoplasms (AONs) are notably rare and typically nonfunctional tumors that are discovered as incidentalomas and exist on a spectrum of malignant potential. CASE PRESENTATION: We discovered an exceptionally large (15 cm in the greatest dimension) incidentaloma in a 73-year-old man with left back pain and he was treated with robotic-assisted adrenalectomy. Surgical pathology was consistent with AON of borderline uncertain malignant potential; adjuvant mitotane and radiation were omitted based on shared decision-making. CONCLUSION: Large AONs are rare, usually benign tumors that can be safely treated with robotic-assisted adrenalectomy. Surgical pathology is the crux of diagnosis and post-operative management, as it informs both the initiation of adjuvant therapy and the stringency of post-operative surveillance.

Reliability and Management Outcomes Following a Percutaneous Biopsy Diagnosis of Oncocytoma: A 15-year Retrospective Analysis.

Background There is uncertainty in the management of renal masses diagnosed as oncocytomas with image-guided percutaneous biopsy. Purpose To assess the reliability of a diagnosis of oncocytoma based on image-guided percutaneous renal mass biopsy and evaluate patient outcomes following different management strategies. Materials and Methods In this retrospective study, image-guided percutaneous biopsy pathology reports from April 2004 to April 2019 were searched for keywords "oncocytoma" and "oncocytic neoplasm" and compared with surgical pathology or repeat biopsy results. Patients with at least 12 months of clinical follow-up and known cause of death were grouped according to management strategies, and disease-specific survival and metastatic renal cell carcinoma (RCC)-free survival were compared. Mass growth rates were calculated with use of a normal linear mixed model. Results The database yielded 160 biopsy reports of 149 renal masses in 139 patients; 149 masses were categorized as oncocytoma (n = 107), likely oncocytoma (n = 12), oncocytic neoplasm (n = 28), and indeterminate with oncocytoma in differential (n = 2). Biopsied masses categorized as oncocytoma or likely oncocytoma were oncocytomas in 16 of 17 masses (94%) based on surgical pathology or repeat biopsy; four of eight masses (50%) categorized as oncocytic neoplasms were low-grade RCCs. Outcome analysis included 121 patients (mean age ± SD, 68 years ± 9.1; 82 men); 80 patients initially underwent active surveillance (11 were later treated), 33 underwent ablation, and eight underwent surgery. Disease-specific survival and metastatic-free survival were 100% after each management strategy (median follow-up, 86.6 months; range, 14.2-207.9 months). Mass growth rate (mean, 1.7 mm per year) showed no evidence of a significant difference among biopsy result categories (P = .37) or initial (P = .84) or final management strategies (P = .11). Conclusion Image-guided percutaneous biopsy diagnosis of renal oncocytoma was reliable. Although some masses diagnosed as oncocytic neoplasms were low-grade renal cell carcinomas (RCCs) at final diagnosis, no patients died of RCC, including those managed with active surveillance. © RSNA, 2023 See also the editorial by Lockhart in this issue.

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.

No difference in renal function outcomes for patients with oncocytoma managed with active surveillance vs. partial nephrectomy.

OBJECTIVES: To investigate the difference in renal function outcomes for patients with oncocytomas undergoing active surveillance (AS) vs. partial nephrectomy (PN). METHODS: We reviewed our institutional database for patients with biopsy/surgically confirmed oncocytoma from 2000-2020. The primary outcome was to assess for differences in renal function outcomes in patients undergoing AS vs. PN. We fit two generalized estimating equation (GEE) with an interaction term between follow up time and management strategy to predict 1) mean eGFR for patients managed with AS and PN and 2) the probability of progression to CKD stage III or greater. RESULTS: We identified 114 eligible patients, of which 32 were managed with AS. Median follow-up was 21 months vs. 44 months for PN vs. AS patients. AS patients tended to be older (median: 72 years vs. 65 years, P<0.001) and have lower baseline renal function (median: eGFR: 71 mL/min/1.73m2 vs. 82 mL/min/1.73m2, P<0.001) compared with PN patients. Renal mass size from baseline imaging was similar between patients undergoing PN vs. AS (2.8 cm vs. 2.9 cm, P=0.634). For patients undergoing PN vs. AS, there was not a significant difference in predicted longitudinal eGFR (-0.079, 95% CI -0.18-0.023, P=0.129) or predicted probability of progression to CKD stage III or greater (OR: 0.61, 95% CI: 0.16-2.33, P=0.47). CONCLUSIONS: In our institutional dataset, patients undergoing AS or PN with an oncocytoma had similar long-term renal function outcomes. Given similar renal function outcomes in patients undergoing AS and PN, surgery should remain reserved for select patients with oncocytomas.

Characteristics of renal oncocytomas and clinical novelties: Single center experience of 17 years.

OBJECTIVE: To assess the characteristics of renal oncocytomas and the clinical outcomes of patients in the last 17 years in our institution. METHODOLOGY: The medical records of the patients who underwent partial and radical nephrectomy from May 2004 to December 2021 were evaluated retrospectively. Radiology and pathology results were evaluated. Patients diagnosed with oncocytoma after surgery were included in the study. RESULTS: Out of 791 patients who were operated for renal masses, 55 patients with the diagnosis of oncocytoma were included in the study, 17 of them were female. The mean age of the patients was 64.77 ± 10.58 years. Open and laparoscopic methods were applied to patients. Partial nephrectomy was performed in 25 patients (46.2%). It was observed that none of the patients with a mean follow-up of 76 months developed recurrence or death due to oncocytoma. CONCLUSION: Oncocytoma is a benign and rare tumor of the kidney which distinguishing it from malign tumors preoperatively with recent techniques is impossible. Especially in small sized tumors, considering the possibility of oncocytoma, nephron sparing surgery should be preferred in terms of patients' benefit. Further research is needed for the novel imaging techniques and biomarkers proposed to be used in routine use to distinguish oncocytoma.

Giant asymptomatic left renal oncocytoma in a 40-year-old man: a case report.

Renal oncocytoma is a benign tumor that arises from epithelial cells of the distal renal tubules. It is naturally presented with a small-sized mass, and giant oncocytoma is uncommon. Renal oncocytoma is frequently asymptomatic and challenging to distinguish preoperatively from renal cell carcinoma (RCC). We present a 40-year-old man who presented with intermittent abdominal pain in the last two years. Abdominal computed tomography (CT) scan showed a large, heterogenous left renal mass measured 15 x 16 x 19.5 cm and associated with central calcifications suspected of RCC. The patient underwent a left radical nephrectomy without complication. The histopathological study revealed typical oncocytoma features. There was no detected recurrence or distant metastasis on six months follow-up. In conclusion, it is challenging to distinguish renal oncocytoma from RCC via preoperative radiology images, especially when a giant mass is present. The only histopathology examination of the removed specimen can provide a definitive diagnosis.

Prognostic factors and survival analysis of Hurthle cell carcinoma: A population-based study.

BACKGROUND: Hurthle cell carcinoma is a rare type of differentiated thyroid cancer and historically associated with a worse prognosis. The aim of this study was to define the demographic and socioeconomic factors, tumor characteristics, and surgical treatment status associated with Hurthle cell carcinoma survival using the most recent population-level data. METHODS: The Surveillance, Epidemiology, and End Results database was queried for adult patients (>18 years of age) diagnosed with Hurthle cell carcinoma from 2000 to 2018. The demographic factors, socioeconomic factors, tumor characteristics, and extent of surgery data were collected as potential predictors. The outcomes of interest were 10-year overall and disease-specific survival, which were estimated using the Kaplan-Maier method. The associations between the potential predictors and survival were evaluated using the Cox proportional hazard model. RESULTS: In total, 4,643 patients with Hurthle cell carcinoma were identified using the Surveillance, Epidemiology, and End Results database. The cohort was predominately White, had a mean age of 57.7 (±15.6), 69% female sex, and median follow-up was 90 months. The 10-year overall survival and Hurthle cell carcinoma-specific survival were 78.1% (95% confidence interval: 76.7%-79.5%) and 91.8% (95% confidence interval: 90.9%-92.9%), respectively. Younger age <55 years, female sex, White race, Hispanic ethnicity, higher household income, and lower tumor grade and stage were significantly associated with increased survival (P < .01). In the multivariate Cox proportional hazard model, all variables except race and ethnicity remained significantly associated with overall survival. Although patients who underwent thyroid surgery had improved survival compared to no surgery, the extent of surgery did not have any effect on their overall or disease-specific survival. CONCLUSION: This study highlighted the aggressive nature of Hurthle cell carcinoma and the effect of socioeconomic factors, such as household income, which may play a role in Hurthle cell carcinoma survivorship. Research is needed to understand the interplay of these factors and their role in predicting patient outcomes.

Renal tumor biopsy in patients with cT1b-T4-M0 disease susceptible to radical nephrectomy: analysis of safety, accuracy and clinical impact on definitive management.

PURPOSE: Renal tumor biopsy was provided in patients candidate to radical nephrectomy for a renal mass ≥4 cm, to evaluate treatment deviation. METHODS: Between 2008 and 2017, 102 patients with a solid renal mass ≥4 cm with no distant metastases underwent preliminary renal tumor biopsy. We investigated the proportion of patients who proceeded with radical nephrectomy, variables predicting non-renal cell carcinoma (RCC) and concordance between biopsy findings and definitive pathology. RESULTS: Median tumor size was 70 mm (IQR 55-110). Clinical stage was cT1b in 41, cT2 in 33, cT3 in 25 and cT4 in three patients. A median of three (IQR 2-3) renal tumor biopsies were taken with 16/18 Gauge needles in 97% of cases. Clavien grade I complications occurred in five cases. Malignant tumors were documented in 84 patients: 78 RCCs and six non-RCCs. Fifteen biopsies documented oncocytoma and three were non-diagnostic. Grade was reported in 50 RCCs: 42 (84%) were low and eight (16%) high grade. Eighty-three patients proceeded with radical nephrectomy; six non-RCC malignant tumors underwent combined and/or intensified treatment; 13 of 15 patients with oncocytoma did not undergo radical nephrectomy (eight underwent observation). Definitive pathology confirmed diagnosis in all cases. Grade concordance was 84%, considering two tiers (high vs low grade). No preoperative clinical variable predicted definitive pathology. CONCLUSIONS: Renal tumor biopsy is a safe procedure that leads to radical nephrectomy in most tumors ≥4 cm. Nonetheless, 20% of patients exhibited non-RCC histology. Renal tumor biopsy should be considered in this setting.

Central Stellated Scar Sign: Classic Imaging Finding of Renal Oncocytoma with Radiopathological Correlation

Oncocytomas are benign tumors, with the classic stellate scar found in up to a third of cases, mainly in large ones. We present the case of a 69-year-old man with a palpable, mobile, and painless mass on the right flank, where abdominal ultrasound and abdominal computed tomography detected the presence of a solid right kidney lesion with a central stellate area in the sinus. Postoperative histological analysis confirmed the presence of oncocytoma with a central stellate scar. Biopsy may not be useful to distinguish this entity from renal carcinoma, surgery being necessary for definitive confirmation of the diagnosis of oncocytoma (AU)

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.

A Rare Case of Synchronous Oncocytoma and Angiomyolipoma of the Kidney.

BACKGROUND The co-occurrence of renal oncocytoma and angiomyolipoma is exceedingly rare. To date, 17 such cases have been reported in the literature. This report describes a unique case of that association that presented as a single renal mass on imaging. CASE REPORT A 75-year-old woman presented with epigastric discomfort. A CT scan of the abdomen revealed a 6.6×5.7×4.7 cm enhancing right renal mass. Gross examination revealed a nodular, well-circumscribed, tan-brown mass located in the lower pole of the kidney that was abutting the renal capsule. Interestingly, superior to this mass, there was an adjacent, pale tan-white, firm, well-circumscribed nodule in the mid-pole, which was not detected on the CT scan and grossly extended to 1.1 cm of the overlying renal capsule. Histologically, the larger tumor showed characteristic features of oncocytoma. The smaller tumor had an admixture of mature adipose tissue, smooth muscle, and vessels, consistent with a renal angiomyolipoma. CONCLUSIONS We present a new case of synchronous renal angiomyolipoma and oncocytoma, which were uniquely adjacent and coexisted with minimal intermingling renal parenchyma. Other "eosinophilic renal tumors" are significant differential diagnosis considerations. Due to the close proximity of these lesions, this association can present clinically and radiologically as a single renal mass. Careful examination of the nephrectomy specimen is essential for the proper detection of small-sized tumors.

Granular Cell Tumor and Spindle Cell Oncocytoma of the Pituitary Gland: Imaging and Intraoperative Cytology Diagnostic Dilemmas and Management Challenges.

BACKGROUND: Tumors arising from the posterior pituitary gland are rare and closely resemble pituitary adenoma in presentation and imaging. Most of them come as a histopathologic surprise. We have analyzed the posterior pituitary tumors managed in our institute and have discussed the dilemmas in imaging, challenges in intraoperative squash cytology, and surgical management. METHODS: We retrospectively reviewed our operative database of pituitary tumors over the past 10 years, which included five posterior pituitary tumors (three granular cell tumors [GCTs] and two spindle cell oncocytomas [SCOs]). Clinical, imaging, and endocrine characteristics; intraoperative details; histopathologic features; and postoperative outcomes were collected and analyzed. RESULTS: The mean age of the patients was 47 years. All patients presented with varying degrees of vision loss. Radiology revealed a sellar / suprasellar lesion with the pituitary gland seen separately in two of three GCTs, whereas a separate pituitary gland could not be identified in both the SCOs. Pituitary adenoma was a radiologic diagnosis in only two of five cases. Three patients underwent a transsphenoidal surgery, whereas two underwent surgery by the transcranial approach. Intraoperative cytology was challenging, though a possibility of posterior pituitary tumor was considered in three of four cases, whereas one was considered meningioma. All the tumors were very vascular and influenced the extent of resection. CONCLUSIONS: GCTs and SCOs are relatively uncommon tumors that are difficult to diagnose on preoperative imaging. Intraoperative squash cytology too can pose challenges. A preoperative suspicion can prepare the surgeon for surgery of these hypervascular tumors. The transcranial approach may be necessary in cases of uncertainty in imaging.