Renal glomus tumor (GT) is a rare neoplasm originating from glomus cells within the kidney. The subtype designated as uncertain malignant potential (UMP) is particularly rare, with limited literature available. Its clinical presentation and imaging features closely resemble those of renal cell carcinoma (RCC), making accurate preoperative diagnosis difficult. This case report aims to describe the clinical, radiological, and pathological features of a renal GT of UMP and to summarize previously reported cases to improve recognition of this rare entity. We report a case involving a 36-year-old male who was incidentally found to have a cystic-solid mass (5.8 × 5.1 × 4.2 cm) in the lower pole of the right kidney during evaluation for tinnitus. Contrast-enhanced CT suggested a diagnosis of RCC, prompting laparoscopic partial nephrectomy. Postoperative pathology revealed focal mild cellular atypia and a Ki-67 proliferation index of approximately 10%. Immunohistochemistry showed positivity for SMA and Collagen IV, focal positivity for CD34, and negativity for CD10, HMB45, Melan-A, Desmin, CK, and CK7. Based on histomorphology and immunoprofile, a diagnosis of primary renal GT with UMP was established. The patient recovered well postoperatively without further treatment, and no local recurrence or distant metastasis was observed during an 8-month follow-up. We reviewed the existing literature to summarize the clinical characteristics and diagnostic considerations of UMP-type renal GT, aiming to enhance recognition of this rare entity.
Primary renal mesenchymal tumors (PRMT) are uncommon, heterogeneous, and diagnostically challenging neoplasms. Comprehensive institutional data on the prevalence, clinicopathological features, immunohistochemical and molecular profiling, and outcomes of PRMTs in South Asia are limited. This retrospective study analyzed 64 surgically resected PRMTs from 1975 nephrectomies performed at our institution between January 2014 and June 2024. Tumors were categorized according to the World Health Organization (WHO) 2022 classification system. Clinico-radiological and histopathological, treatment and follow-up data were retrieved from the hospital information systems. Among all surgical nephrectomies, 64 PRMTs were identified (prevalence 3.2%). According to the WHO 2022 classification, 37 tumors (58%) were benign, 22 (34%) were malignant, and 5 tumors (8%) were intermediate. The overall female-to-male ratio was 1.1:1. Adults comprised 81.2% of the patients (n = 52), children comprised 18.8% (n = 12). Angiomyolipoma was the most common tumor, comprising 56% of PRMTs (n = 36), followed by clear cell sarcoma of the kidney (n = 7; 11%), Ewing sarcoma (n = 6; 9%), leiomyosarcoma (n = 6; 9%), congenital mesoblastic nephroma (n = 3; 5%), and other rare entities. The mean follow-up duration was 41.6 months, with recurrence in three cases and metastasis in six cases. This is one of the largest Indian series conducted on PRMTs, documenting a diverse spectrum. Malignant tumors tend to occur at a younger age, although exceptions have been reported. The occurrence of extremely rare entities highlights the critical role of immunohistochemistry and molecular testing in the accurate classification and treatment planning of these tumors.
Immune checkpoint inhibitor-based combinations are the current standard first-line treatment for metastatic renal cell carcinoma (mRCC). However, tyrosine kinase inhibitors remain the predominant first-line therapy in many low- and middle-income countries because of limited affordability and access to immunotherapy. Dose reduction of tyrosine kinase inhibitors is frequently practiced in real-world settings to improve tolerability and treatment adherence. However, data on outcomes with reduced-dose pazopanib and subsequent treatment patterns remain limited. This retrospective study evaluated the effectiveness and safety of reduced-dose pazopanib (400 mg daily) as first-line systemic therapy in anti-VEGF treatment-naive patients with mRCC who were unable to access immunotherapy. Patients aged ≥18 years with histologically confirmed RCC who received reduced-dose pazopanib between January 2016 and December 2023 were included. Data on response rates, toxicities, and subsequent treatment lines were collected from hospital records. Survival outcomes were estimated using the Kaplan–Meier method. Eighty-four patients received reduced-dose pazopanib during the study period. The median age was 57.5 years (range, 19–76 years). Most patients were male (79%) and had clear-cell histology (79.7%). Sixty-six per cent of patients presented with de novo metastatic disease, while 52% belonged to the International Metastatic RCC Database Consortium (IMDC) intermediate-risk group. Nearly half of the cohort (45.1%) had an Eastern Cooperative Oncology Group (ECOG) performance status ≥2. Nephrectomy was performed in 52 patients (61.9%); of these, radical nephrectomy in 29 (58%) and cytoreductive nephrectomy in 23 (42%). Clinical benefit at the first response assessment was observed in 69 patients (82.1%). At a median follow-up of 41.3 months, median progression-free survival and overall survival were 8.9 months (95% CI, 6.7–11.0 months) and 28.6 months (95% CI, 19.0–38.1 months), respectively. The most common adverse events were skin and hair depigmentation (35.7%), diarrhea (25%), hand–foot syndrome (21.4%), and hypertension (14.3%). Grade 3–4 adverse events occurred in 5.9% of patients, and treatment discontinuation due to toxicity occurred in 2.4%. No treatment-related deaths were reported. Reduced-dose pazopanib appears to be a feasible first-line treatment option for anti-VEGF treatment-naïve patients with mRCC who are unable to access immunotherapy, providing clinically meaningful efficacy with a favorable toxicity profile.
Primary Non-Hodgkin's Lymphoma (NHL) involving the adrenal gland is rare, and its synchronous occurrence with renal cell carcinoma (RCC) is exceedingly uncommon. Although adrenal involvement is observed in approximately 20% of systemic NHL cases, primary adrenal lymphoma accounts for only a small proportion of these cases. RCC accounts for 2-3% of all malignancies and is the second most common genitourinary cancer. The coexistence of RCC with other primary malignancies has been described, often in the context of familial syndromes; however, its synchronous presentation with primary adrenal NHL is exceptionally rare. A 54-year-old male with diabetes presented with intermittent dull left flank pain for 18 months and a recent loss of appetite. Contrast-enhanced computed tomography revealed a large, heterogeneous, hyper-enhancing mass in the left suprarenal region replacing the adrenal gland, along with a separate enhancing exophytic lesion in the left kidney. Positron emission tomography showed high metabolic activity in both lesions and regional lymph nodes. The patient underwent open radical nephrectomy with adrenalectomy and regional lymph node dissection. Histopathology demonstrated diffuse large B-cell lymphoma involving the adrenal gland with extension into the kidney, perinephric tissue, and perihilar fat, along with a separate Grade II clear cell RCC in the lower pole of the kidney. Immunohistochemistry confirmed B-cell lineage lymphoma. Lymph nodes were negative for metastasis. The patient received nine cycles of CHOP (Cyclophosphamide, Doxorubicin [Hydroxydaunorubicin], Vincristine [Oncovin], and Prednisolone) chemotherapy and remains recurrence-free at the 6-month follow-up. This case highlights a rare synchronous occurrence of primary adrenal NHL and ipsilateral clear cell RCC. Shared genetic or immunological mechanisms may underlie this association, warranting further investigation.
Primary Non-Hodgkin’s Lymphoma (NHL) involving the adrenal gland is rare, and its synchronous occurrence with renal cell carcinoma (RCC) is exceedingly uncommon. Although adrenal involvement is observed in approximately 20% of systemic NHL cases, primary adrenal lymphoma accounts for only a small proportion of these cases. RCC accounts for 2–3% of all malignancies and is the second most common genitourinary cancer. The coexistence of RCC with other primary malignancies has been described, often in the context of familial syndromes; however, its synchronous presentation with primary adrenal NHL is exceptionally rare. A 54-year-old male with diabetes presented with intermittent dull left flank pain for 18 months and a recent loss of appetite. Contrast-enhanced computed tomography revealed a large, heterogeneous, hyper-enhancing mass in the left suprarenal region replacing the adrenal gland, along with a separate enhancing exophytic lesion in the left kidney. Positron emission tomography showed high metabolic activity in both lesions and regional lymph nodes. The patient underwent open radical nephrectomy with adrenalectomy and regional lymph node dissection. Histopathology demonstrated diffuse large B-cell lymphoma involving the adrenal gland with extension into the kidney, perinephric tissue, and perihilar fat, along with a separate Grade II clear cell RCC in the lower pole of the kidney. Immunohistochemistry confirmed B-cell lineage lymphoma. Lymph nodes were negative for metastasis. The patient received nine cycles of CHOP (Cyclophosphamide, Doxorubicin [Hydroxydaunorubicin], Vincristine [Oncovin], and Prednisolone) chemotherapy and remains recurrence-free at the 6-month follow-up. This case highlights a rare synchronous occurrence of primary adrenal NHL and ipsilateral clear cell RCC. Shared genetic or immunological mechanisms may underlie this association, warranting further investigation.
