Non-clear cell renal cell carcinoma (non-ccRCC) is a highly heterogeneous disease group, accounting for approximately 25% of all RCC cases. Due to its rarity and especially heterogeneity, phase III trial data is limited and treatment options generally follow those of clear cell RCC. In the literature, there exists a number of studies with sunitinib, cabozantinib, and everolimus, but data on the efficacy of pazopanib are limited. Our aim in this study was to compare the efficacy of pazopanib and sunitinib, in a multicenter retrospective cohort of non-ccRCC patients. Our study included patients diagnosed with non-ccRCC who received pazopanib or sunitinib treatment as first-line therapy from 22 tertiary hospitals. We compared the progression-free survival (PFS), overall survival (OS), and response rates of pazopanib and sunitinib treatments. Additionally, we investigated prognostic factors in non-ccRCC. PFS and response rates of sunitinib and pazopanib were found to be similar, while a numerical difference was observed in OS. Being 65 years and older, being in the intermediate or poor risk group according to the International Metastatic Renal Cell Carcinoma Database Consortium, having liver metastases, presence of a sarcomatoid component, and having de novo metastatic disease were found to be significantly associated with shorter PFS. Pazopanib treatment appears to have similar efficacy in the treatment of non-ccRCC compared to sunitinib. Though randomized controlled trials are lacking and will probably be never be available, we suggest that pazopanib could be a preferred agent like sunitinib and cabozantinib.
Pazopanib and sunitinib treatments show similar progression free survival, overall survival and objective response rate.IMDC risk group, liver metastasis, sarcomatoid component and de novo metastatic disease were determined as prognostic factors.

Nephroblastoma or Wilms\' tumor (WT) is the most common pediatric renal malignancy but rare in adults. Treatment protocols for adults are typically extrapolated from pediatric guidelines, but there are no standard guidelines for adults due to the rarity of the disease. However, next-generation sequencing has led to new therapeutic options for adult WT patients. We present the first case to our knowledge of a recurrent adult WT treated with dual BRAF/MEK-targeted therapy, which showed initial robust clinical response and was well tolerated.

Soft tissue sarcomas account for less than 1% of new cancer diagnoses, approximately one in five of which are liposarcomas. These tumors typically arise in the deep tissues of the proximal extremity or retroperitoneum, with just under 3% presenting as primary intrathoracic neoplasms. We present an exceedingly rare and particularly unique presentation of primary lung liposarcoma which traversed the mediastinum into the contralateral hemithorax. This report highlights the primary characteristics of the disease and underscores the importance of a multidisciplinary approach to its successful treatment.

OBJECTIVE: Mitotane (Lysodren, o,p\'-DDD [1-(o-chlorophenyl)-1-(p-chlorophenyl)-2,2-dichloroethane)] is currently the only United States Food and Drug Administration and European Medicines Agency-approved product for the treatment of adrenocortical carcinoma.
METHODS: Mitotane is challenging to administer; however, its toxicities (specifically adrenal insufficiency) are well known, and the management of adverse consequences has established approaches. While often viewed through the prism of a cytotoxic agent, it can also interfere with hormone production making it a valuable asset in managing functional ACC. A recently completed prospective trial has shed some light on its use in the adjuvant setting, but further clarity is needed. Many think mitotane has a role in the advanced or metastatic setting, although prospective data are lacking and retrospective analyses are often difficult to interpret.
CONCLUSIONS: When used carefully and thoughtfully, especially in patients with hormonal excess, mitotane is an important component of the treatment armamentarium for ACC.

Ossifying fibromyxoid tumour (OFMT) is a rare subcutaneous soft tissue neoplasm, with unclear lineage and intermediate differentiation. Typically presenting as a benign growth, however it can recur locally, and malignant variants have been reported. We present an unusual case of OFMT occurring as a subcutaneous mass on the right upper back. A 29-year-old gentleman presented with one-year history of a painless, slowly enlarging mass on his right upper back. He had no relevant medical history, was not on any medications, and had no family history of skin cancer. Physical examination revealed a 25mm x 25mm skin-coloured, stony-hard, well circumscribed mass. The initial clinical diagnosis was a calcified epidermoid cyst. The lesion was excised and sent for histopathology, which revealed well-circumscribed cellular spindle cells with low mitotic index. Immunohistochemistry showed variable S100 positivity. Due to its rarity, the diagnosis was delayed as the biopsy required a second opinion from a tertiary hospital. The final pathological diagnosis confirmed OFMT. This case describes the very rare presentation of OFMT in a dermatology clinic, highlighting the importance of recognising this neoplasm due to its potential for recurrence and metastasis. This unusual case of OFMT broadens the dermatological differential diagnosis for a subcutaneous mass.

Very rare tumors are usually divided into two groups: One includes tumors that are rare among both children and adults; the other one encompasses tumors that frequently occur in adults but are rarely observed in children. In this review, we focus on adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas, with special attention to the role of surgery as main curative intervention or as part of the multimodal treatment.

Background: Large population-based registries, such as the Surveillance, Epidemiology and End Results (SEER) Registry, help in the study of rare tumors, including medullary thyroid cancer (MTC), but lack data to understand the natural history of the disease. The Medullary Thyroid Cancer Collaborative Registry (MTCCoRe) is an exhaustive multi-institutional collection of demographic, clinical, and pathological data. To determine the extent to which MTCCoRe represents the real-world MTC population, we compared the characteristics of patients enrolled in MTCCoRe with patients enrolled in population-based cancer registries. Methods: Comparison of demographic and clinical characteristics of MTC patients who were enrolled in MTCCoRe, Texas Cancer Registry (TCR), California Cancer Registry (CCR), and SEER between 1995 and 2018. Results: A total of 1416 patients were identified in MTCCoRe, 329 in TCR, 2105 in CCR, and 3820 in SEER. Percentages of patients 20-54 years in MTCCoRe were 58.0%, 50.2% in TCR, 47.2% in CCR, and 44.8% in SEER (p < 0.0001). About half of the patients were female (55.9% in MTCCoRe, 61.4% in TCR, 59% in CCR, and 57.5% in SEER (p = 0.3). Percentages of Hispanic and Black patients differed among cohorts (10.1% and 3.8% for MTCCoRe, 23.7% and 8.2% for TCR, 24.8% and 4.9% in CCR, and 15.9% and 8.2% for SEER, respectively; p < 0.001). MTCCoRe patients presented with more advanced T and N classifications than patients in the other registries (MTCCoRe, 28.6% T3-4 and 49.4% N1; TCR, 12.7% and 32.2%; CCR, 18.6% and 32.4%; and SEER, 24% and 37.8%; p < 0.0001). Prevalence of M1 disease was 10% in MTCCoRe, 11.9% in TCR, 14.1% in CCR, and 9.5% in SEER (p < 0.0001). In the MTCCoRe, 11.4% underwent systemic therapy (compared with 0.3% in TCR and 5.6% in CCR). Conclusions: The clinicodemographic profile of patients with MTC enrolled in a multi-institutional registry differs from those enrolled in population-based databases, with lower proportions of Hispanic and Black patients but additive data on treatment modalities. Moving forward, MTCCoRe and other registry and clinical trial enrollment efforts should intentionally include underrepresented groups via community engagement techniques, patient stakeholder involvement, and inclusion of languages other than English in study materials to yield more generalizable results and conclusions.

Lipomas are benign soft tissue tumors that are ubiquitous in nature. Available literature suggests that benign tumors are harmless unless they increase in size, resulting in compression of vital structures. This case report discusses the case of a 52-year-old man who presented to the clinic with a painless, growing lump on the right side of his mouth. The patient\'s symptoms included difficulty swallowing and speaking, which led the doctors to recommend surgical excision of the mass. There were no issues during the mass removal surgery, and the incision healed without compromising the lingual or hypoglossal nerves or Wharton\'s duct, as observed during follow-up visits. Patient history, symptoms, preoperative examination, treatment strategy, and surgical technique are all included in this case study, which focuses on the extremely unusual development of lipomas in the oral cavity, particularly on the floor of the mouth.

Sino-nasal respiratory epithelial adenomatoid hamartomas (REAHs) are rare entity. They are benign tumors with excellent results after complete excision. We report a case of a 57-year-old male with a history of endoscopic surgery for right nasal polyps 20 years ago. The patient presented nasal obstruction that persisted for 10 years without anosmia nor epistaxis. Nasal endoscopy found a tissular mass filling the right nasal cavity extending to the nasopharynx. CT scan and MRI demonstrated soft tissue opacification of the right maxillary sinus and the homolateral anterior ethmoid cells with extension to the nasal cavity. The suspected diagnosis on imaging was an Inverted papilloma with a wide implantation base on the posterior part of the nasal septum. No endocranial or orbital extension was noted. The patient underwent endoscopic sinus surgery with complete extirpation of the tumor and a right ethmoidectomy. Histopathological assessment showed features consistent with REAH. No recurrence was noted at 1 year follow-up.

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