RESUMEN
Xanthelasma palpebrarum is one of the most common cutaneous xanthomas in humans. Currently, there are various methods available for treating xanthelasma palpebrarum, but the high treatment frequency and recurrence rate remain significant challenges for patients. Therefore, it is necessary to establish a reasonable and effective clinical grading system to guide the diagnosis and treatment of xanthelasma palpebrarum. We developed a clinical scoring system related to local injection of pingyangmycin for the treatment of xanthelasma palpebrarum, which can be used to predict early prognosis and treatment outcomes in patients. We collected and retrospectively studied 246 outpatient cases of xanthelasma palpebrarum treated with local injection of pingyangmycin in the Department of Plastic Surgery at Shanghai East Hospital from February 2020 to August 2022. Potential independent risk factors for adverse outcomes (recurrence or non-recurrence) were considered in univariate and multivariate logistic regression models. Predictive factors were determined based on the multivariate logistic regression model and Cox model, and a scoring grading system was established. External validation was conducted on an independent cohort of 110 patients. Based on logistic regression analysis, the number, area, and color of lesions were identified as significant predictive indicators (P < 0.05), with respective AUCs of 0.710, 0.799, and 0.755. The Cox model established hazard ratios for four new severity indicators of xanthelasma palpebrarum: hyperlipidemia, number of lesions, lesion area, and lesion grayscale value. Based on these findings, a new clinical grading model was developed, which was validated to be effective in the external cohort. The new scoring-based clinical predictive model can effectively predict the number of pingyangmycin injection treatments and prognosis in patients with xanthelasma palpebrarum. It holds promise for broader application in clinical practice.
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Enfermedades de los Párpados , Xantomatosis , Humanos , Xantomatosis/diagnóstico , Xantomatosis/patología , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto , Pronóstico , Enfermedades de los Párpados/diagnóstico , Enfermedades de los Párpados/tratamiento farmacológico , Bleomicina/administración & dosificación , Resultado del Tratamiento , Anciano , Recurrencia , China/epidemiología , Índice de Severidad de la Enfermedad , Adulto Joven , Factores de Riesgo , Párpados/patologíaRESUMEN
Xanthoma typically occurs in the subcutaneous tissues, with rare cases of xanthoma in the joints. However, the case of knee joint osteonecrosis combined with xanthoma is even more uncommon. In this article, we described a 50-year-old female patient who suffered xanthoma in the knee joint on the basis of osteonecrosis of the knee joint. The primary clinical symptoms were knee joint pain and limited mobility. The patient initially received conventional treatment for osteonecrosis. However, there was no significant improvement. Later, we found a synovial xanthoma in the patient's knee. Finally, she underwent arthroscopic excision of the knee joint synovial xanthoma. Following the procedure, her VAS score decreased from 7 to 2, and knee joint mobility increased from 10-103° to 10-140°. Through our follow-up, the patient did not exhibit symptom recurrence. This case is valuable as it provides a feasible therapeutic approach for future clinical applications.
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Artroscopía , Articulación de la Rodilla , Osteonecrosis , Xantomatosis , Humanos , Femenino , Persona de Mediana Edad , Articulación de la Rodilla/cirugía , Articulación de la Rodilla/diagnóstico por imagen , Osteonecrosis/cirugía , Osteonecrosis/diagnóstico por imagen , Osteonecrosis/complicaciones , Osteonecrosis/etiología , Xantomatosis/cirugía , Xantomatosis/complicaciones , Xantomatosis/diagnóstico , Resultado del Tratamiento , Rango del Movimiento Articular , Imagen por Resonancia MagnéticaRESUMEN
Xanthogranulomatous cholecystitis is a rare chronic inflammatory disease of the gallbladder associated with complications such as perforation, dense adhesions and fistulation. We present a case of a female patient in her 20s who presented with three episodes of recurrent cholecystitis over 3 months. Her medical history included an endoscopic retrograde cholangiopancreatography for obstructive jaundice due to choledocholithiasis. As there was no possibility of performing an urgent cholecystectomy during her initial admission, she was listed for an elective operation. Laparoscopic inspection revealed a pericholecystic mass involving the omentum, transverse colon, duodenum and liver and findings suspicious of a cholecystoappendiceal fistula. Laparoscopic cholecystectomy and appendicectomy were performed. Early index admission laparoscopic cholecystectomy should be performed in patients with acute cholecystitis to prevent higher grades of operative difficulty and associated complications. This case presentation-cholecystoappendiceal fistula associated with xanthogranulomatous cholecystitis-emphasises the necessity of complying with national guidelines in managing acute calculous cholecystitis.
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Colecistectomía Laparoscópica , Colecistitis , Xantomatosis , Adulto , Femenino , Humanos , Apendicectomía/métodos , Fístula Biliar/diagnóstico , Fístula Biliar/etiología , Fístula Biliar/cirugía , Colecistectomía Laparoscópica/métodos , Colecistitis/complicaciones , Colecistitis/diagnóstico , Colecistitis/cirugía , Fístula Intestinal/diagnóstico , Fístula Intestinal/etiología , Fístula Intestinal/cirugía , Epiplón/patología , Xantomatosis/complicaciones , Xantomatosis/cirugía , Xantomatosis/diagnósticoAsunto(s)
Xantomatosis , Humanos , Xantomatosis/diagnóstico , Xantomatosis/patología , Masculino , FemeninoRESUMEN
Adipocytic tumors are mesenchymal tumors that are commonly reported in psittacine birds; however, large-scale studies evaluating their prevalence and associated risk factors are lacking. A retrospective study of adipocytic tumors in psittacine birds was performed by reviewing pathology submissions from the University of California, Davis-Drury Reavill Pathology Database, containing 26 013 submissions from psittacine birds (1998-2018). Age, sex, genus, anatomic distribution, and pathological diagnosis were collected for each case when available. The prevalence, risk factors, and association with other lipid-accumulation disorders were reported. A total of 450 cases of lipoma, 129 cases of myelolipoma, 35 cases of hemangiolipoma, 31 cases of liposarcoma, and 451 cases of xanthoma were identified. The prevalence of adipocytic tumors and xanthomas on necropsy was 1.3% (158/11 737, 95% confidence interval [CI]: 1.1-1.6). Adipocytic tumors were identified in 27 genera. Amazona (odds ratio [OR] = 1.93, 95% CI: 1.24-2.99, p = 0.004), Myiopsitta (OR = 2.3, 95% CI: 1.0-5.2, p = 0.041), Melopsittacus (OR = 3.4, 95% CI: 2.1-5.5, p < 0.001), and Agapornis (OR = 3.5, 95% CI: 2.0-6.1, p < 0.001) had significantly higher odds of developing adipocytic tumors compared with other genera, whereas Ara had significantly lower odds (OR = 0.5, 95% CI: 0.3-0.9, p = 0.030). Age was also a significant risk factor for many types of adipocytic tumors. There was no significant association between general adipocytic tumor formation and atherosclerosis or hepatic lipidosis. Xanthomas were associated with atherosclerosis (OR = 1.88, 95% CI: 1.01-3.51, p = 0.048), but not hepatic lipidosis (p = 0.503). On necropsy, the trunk and air sacs were the most common sites of xanthoma formation, whereas the trunk and liver were the most common sites of lipoma and myelolipoma formation, respectively.
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Enfermedades de las Aves , Psittaciformes , Xantomatosis , Animales , Enfermedades de las Aves/epidemiología , Enfermedades de las Aves/patología , Xantomatosis/veterinaria , Xantomatosis/epidemiología , Xantomatosis/patología , Factores de Riesgo , Prevalencia , Estudios Retrospectivos , Masculino , FemeninoRESUMEN
BACKGROUND: Sitosterolemia, an autosomal recessive condition, is characterized by impaired metabolism of plant sterols. Clinical symptoms include skin xanthoma, premature atherosclerotic disease, arthritis, and unexplained hematological abnormalities. However, there is a dearth of studies on sitosterolemia-related brain damage. METHODS: This study focused on the family of two sitosterolemia patients who presented with severe hypercholesterolemia and xanthoma. Radiological examinations, biopsies, whole-exome sequencing (WES), and plant sterol tests were conducted. RESULTS: The index patient, a 66-year-old female, initially exhibited weakness in both lower limbs and later developed urinary and fecal incontinence. Neuroimaging showed that the falx of the brain had irregular fusiform thickening. Significant tissue edema was observed around the lesions in the bilateral frontal-parietal lobes. Pathological analysis of the biopsied brain lesion revealed extensive cholesterol crystal deposition and lymphocyte infiltration in the matrix. The index patient who experienced cerebral impairment and her sister both carried two compound heterozygous variants in ATP binding cassette transporter G5 (ABCG5). These included the nonsense variants NM_022436: c.751 C > T (p.Q251X) in exon 6 and NM_022436: c.1336 C > T (p.R446X) in exon 10. A notable increase in plant sterol levels was observed in the younger sister of the index patient. CONCLUSION: This study highlights a previously unreported neurological aspect of sitosterolemia. Imaging and pathology findings suggest that cholesterol crystals may be deposited in connective tissues such as the cerebral falx and pia mater through blood circulation.
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Transportador de Casetes de Unión a ATP, Subfamilia G, Miembro 5 , Hipercolesterolemia , Enfermedades Intestinales , Errores Innatos del Metabolismo Lipídico , Fitosteroles , Humanos , Femenino , Fitosteroles/efectos adversos , Anciano , Hipercolesterolemia/genética , Hipercolesterolemia/patología , Hipercolesterolemia/complicaciones , Errores Innatos del Metabolismo Lipídico/genética , Errores Innatos del Metabolismo Lipídico/patología , Errores Innatos del Metabolismo Lipídico/diagnóstico por imagen , Enfermedades Intestinales/genética , Enfermedades Intestinales/patología , Enfermedades Intestinales/diagnóstico por imagen , Transportador de Casetes de Unión a ATP, Subfamilia G, Miembro 5/genética , Encéfalo/patología , Encéfalo/diagnóstico por imagen , Secuenciación del Exoma , Xantomatosis/patología , Xantomatosis/genética , Xantomatosis/diagnóstico por imagen , Linaje , Colesterol/sangre , Masculino , Sitoesteroles , LipoproteínasRESUMEN
PURPOSE: Within the large umbrella of histiocytosis are a few similar yet heterogenous entities involving the orbit and periocular tissues with or without systemic infiltration, termed adult onset xanthogranuloma or orbital xanthogranuloma. Due to rarity of these conditions, different classifications in use, diverse clinical presentations and still unknown etiology, the aim of this paper was to provide an up-to-date literature review of the actual understanding of histiocytosis and its subgroups involving the orbit and periocular area, diagnostic strategies and therapeutic modalities. METHODS: We present a review of literature and small case series comprising four patients diagnosed and treated in the period from 2001 until 2023 in our hospital. Clinical files of 4 patients with adult-onset xanthogranulomatous disease of the orbit and ocular adnexa (AOXGD) were reviewed retrospectively. Clinical, laboratory, radiological, histopathological, and immunohistochemical findings were reexamined. RESULTS: Reviewing medical records of our patients with AOXGD, we found significant overlap between histiocytosis and different immune disorders. A broad workup should be considered in these patients as they can harbour severe immune disfunctions and hematologic disorders. Preferred treatment modality depends on a histopathologic type of AOXGD, clinical presentation and systemic involvement and should be conducted multidisciplinary. CONCLUSION: The diagnosis is often delayed because of its rarity and diverse clinical findings. Development of molecular genetic tests, detection of BRAF V600E mutation and different types of kinase mutations, mutations in transcriptional regulatory genes as well as tyrosine kinase receptors have shed a new light on the etiopathogenesis and potential targeted treatment of histiocytosis.
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Enfermedades Orbitales , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Granuloma/diagnóstico , Histiocitosis/diagnóstico , Enfermedades Orbitales/diagnóstico , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Xantomatosis/diagnóstico , AncianoRESUMEN
The oral verruciform xanthoma (OVX) is a rare, benign lesion that occurs predominantly in the masticatory region of the oral cavity. The OVX is small, slow growing, and mostly free of clinical symptoms. The exact pathogenesis is unknown, and a viral etiology such as from a human papillomavirus (HPV) infection has not been proven. Although primarily observed in healthy individuals, there have been cases in patients with autoimmune diseases and with chronic graft-versus-host disease (GvHD). The treatment of choice is complete excision of the lesion. This case report showcases a successful surgical removal of an oral verruciform xanthoma on the left buccal mucosa in a 56-year-old patient with GvHD 14 years after allo-genic stem cell transplantation due to a Non-Hodgkin lymphoma.
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Mejilla , Enfermedad Injerto contra Huésped , Xantomatosis , Humanos , Persona de Mediana Edad , Xantomatosis/diagnóstico , Xantomatosis/cirugía , Enfermedad Injerto contra Huésped/diagnóstico , Enfermedades de la Boca/diagnóstico , Enfermedades de la Boca/cirugía , Enfermedades de la Boca/terapia , Masculino , Mucosa Bucal/patología , Diagnóstico DiferencialRESUMEN
Cerebrotendinous xanthomatosis (CTX) is a rare inherited metabolic disease attributed to the mutation of the gene CYP27A1, resulting in sterol 27-hydroxylase deficiency characterized by deposition of cholestanol and cholesterol in several tissues, like the central nervous system and tendons. Furthermore, cataracts, gallstones, diarrhea and premature atherosclerosis have been reported. Nonetheless, clinical development is extremely heterogeneous in CTX. We report here two cases of CTX genetic alteration in the absence of cholestanol elevation in plasma and tendons but with prominent xanthomas. We propose that CTX may not be characteized by increased plasma cholestanol concentration due to alteration in the sterol 27-hydroxylase gene, but is a more complex pathology where there is significant genetic heterogeneity caused by various CYP27A1 mutations.
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Colestanotriol 26-Monooxigenasa , Colestanol , Colesterol , Tendones , Xantomatosis Cerebrotendinosa , Xantomatosis , Humanos , Xantomatosis Cerebrotendinosa/genética , Xantomatosis Cerebrotendinosa/diagnóstico , Xantomatosis Cerebrotendinosa/sangre , Xantomatosis Cerebrotendinosa/patología , Xantomatosis Cerebrotendinosa/tratamiento farmacológico , Colestanol/sangre , Colesterol/sangre , Colestanotriol 26-Monooxigenasa/genética , Tendones/patología , Femenino , Masculino , Xantomatosis/genética , Xantomatosis/patología , Adulto , Mutación , Persona de Mediana EdadRESUMEN
Adult orbital xanthogranulomatous disease (AOXGD) is a spectrum of histiocytoses with four subtypes. Mitogen-activated protein kinase (MAPK) pathway mutations have been detected in various histiocytic neoplasms, little is known about this in AOXGD. Targeted regions of cancer- and histiocytosis-related genes were analyzed and immunohistochemical staining of phosphorylated ERK (pERK), cyclin D1 and PU.1 was performed in 28 AOXGD and 10 control xanthelasma biopsies to assess MAPK pathway activation. Mutations were detected in 7/28 (25%) patients. Positive staining for pERK and/or cyclin D1 was found across all subtypes in 17/27 (63%) patients of whom 12/17 (71%) did not harbour a mutation. Xanthelasma tissue stained negative for pERK and cyclin D1. Relapse occurred in 5/7 (71%) patients with a MAPK pathway mutation compared to 8/21 (38%) patients in whom no mutation could be detected. Molecular analysis and evaluation for systemic disease is warranted to identify patients at risk of recurrent xanthomatous disease.
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Sistema de Señalización de MAP Quinasas , Mutación , Xantomatosis , Humanos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Sistema de Señalización de MAP Quinasas/genética , Anciano , Xantomatosis/genética , Enfermedades Orbitales/genética , Ciclina D1/genética , Ciclina D1/metabolismo , Adulto Joven , Granuloma/genéticaAsunto(s)
Micosis Fungoide , Neoplasias Cutáneas , Xantomatosis , Humanos , Micosis Fungoide/complicaciones , Micosis Fungoide/patología , Xantomatosis/patología , Xantomatosis/complicaciones , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Masculino , Persona de Mediana Edad , FemeninoRESUMEN
BACKGROUND The gallbladder develops from the hepatic diverticulum during the fourth week of gestation, which also give rise to the liver, extrahepatic biliary ducts, and ventral part of the pancreas. Infrequently, the gallbladder has malformation or disruption in embryogenesis, leading to congenital anomalies. There are various congenital anomalies that can arise in the gallbladder. True or congenital diverticulum of the gallbladder is a rare entity that accounts for only 0.06% of gallbladder congenital anomalies and 0.0008% of cholecystectomies at the Mayo Clinic. CASE REPORT Herein, we report a rare case of a 38-year-old woman who presented to Jubail General Hospital's surgery clinic with right upper-quadrant (RUQ) pain associated with vomiting after meals for 1 month. Laparoscopic cholecystectomy was done and gallbladder tissue was sent to histopathology. Gross examination revealed an outpouching mucosa within the wall that was proven to consist of muscularis and serosa layers under light microscope. Interestingly, xanthogranulomatous inflammation was confined to the diverticulum, unlike the chronic inflammation involving the remaining gallbladder. Based on the above findings, the diagnosis of congenital diverticulum with xanthogranulomatous cholecystitis was made. CONCLUSIONS Gallbladders associated with a true diverticulum are uncommonly found to be buried in the liver, leading to surgical difficulties during cholecystectomy. Therefore, background knowledge of occasional anomalies plays a crucial role in guiding the surgeon to choose the optimal method of management. We also discuss the associated complications that accompany these anomalies, such as non-specific prolonged ailments, acalculous cholecystitis, cholecystitis and cholelithiasis, recurrent cholangitis, and carcinoma of the gallbladder.
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Colecistitis , Divertículo , Vesícula Biliar , Xantomatosis , Humanos , Femenino , Adulto , Xantomatosis/cirugía , Xantomatosis/diagnóstico , Colecistitis/cirugía , Colecistitis/diagnóstico , Divertículo/cirugía , Divertículo/diagnóstico , Divertículo/complicaciones , Vesícula Biliar/anomalías , Vesícula Biliar/patología , Granuloma/cirugía , Granuloma/diagnóstico , Colecistectomía LaparoscópicaRESUMEN
BACKGROUND: A xanthoma is a rare bone condition consisting of a predominant collection of lipid-rich, foamy histiocytes. The central xanthoma of the jaws is a unique benign tumor. CASE REPORT: A 15-year-old Caucasian male has been presented to our department. He had radiological changes in the area of the left mandibular angle, with an area of diffuse osteolysis of 3.0 cm by 2.0 cm. Computed tomography reveals an area of diffuse osteolysis that starts from the distal root of the lower second molar and reaches the ascending process. A bone biopsy was performed, which revealed a benign proliferative process composed of histiocytic cells involving and infiltrating trabecular bone in a background of loose fibrous connective tissue devoid of any other significant inflammatory infiltrate. The size of the formation was 2.9 cm by 2.0 cm. Immunohistochemical staining for CD68 was strongly positive and negative for S-100 and CD1a. From routine blood tests, cholesterol, triglycerides, and blood sugar are within normal values, which excludes systemic metabolic disease. Subsequent to the surgical intervention, the patient underwent postoperative assessments at intervals of 14, 30, 60 days, and a year later, revealing the absence of any discernible complications during the aforementioned observation periods. CONCLUSION: The diagnosis of primary xanthoma of the mandible is rare and can often be confused with other histiocytic lesions. A differential diagnosis should be made with nonossifying fibroma and Langerhans cell histiocytosis, as in our case. In these cases, immunohistochemistry with CD 68, S-100, and CD1a, as well as blood parameters, are crucial for the diagnosis.
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Enfermedades Mandibulares , Xantomatosis , Humanos , Masculino , Adolescente , Xantomatosis/patología , Xantomatosis/diagnóstico , Xantomatosis/cirugía , Enfermedades Mandibulares/patología , Enfermedades Mandibulares/diagnóstico por imagen , Enfermedades Mandibulares/cirugía , Enfermedades Mandibulares/diagnóstico , Tomografía Computarizada por Rayos X , Mandíbula/patología , Mandíbula/diagnóstico por imagen , Mandíbula/cirugía , BiopsiaRESUMEN
Xanthelasma palpebrarum (XP) is the predominant form of cutaneous xanthoma, as it accounts for greater than 95% of cases. It is characterized by the presence of foam cell clusters containing a large amount of low-density lipoprotein (LDL), which are located in the connective tissue of skin, tendons, and fascia. XP lesions commonly present as distinctive yellow-orange macules, papules, or nodules, and are primarily on the upper eyelids as well as the inner canthus. Women are affected twice as often as men, with lesions typically emerging between the ages of 35 and 55. The pathophysiology of XP involves abnormal lipid metabolism and is often associated with hyperlipidemic states like Type II and IV hyperlipidemia, hypothyroidism, weight gain, and fatty diet. Despite the availability of various treatment methods, current XP management lacks standardization, particularly due to limited comparative research. To address this gap, we conducted an extensive literature review of 45 studies published between 2012 to 2023, which provides an updated overview of current XP treatment modalities. This comprehensive analysis will inform researchers and clinicians on the evolving landscape of XP management.
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Enfermedades de los Párpados , Xantomatosis , Humanos , Xantomatosis/terapia , Xantomatosis/diagnóstico , Enfermedades de los Párpados/terapia , Enfermedades de los Párpados/diagnóstico , Enfermedades de los Párpados/metabolismo , Femenino , Párpados/patología , Masculino , AdultoAsunto(s)
Queratinocitos , Xantomatosis , Humanos , Xantomatosis/patología , Queratinocitos/patología , Masculino , Femenino , Metabolismo de los LípidosAsunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/inmunología , Inmunoglobulina G/inmunología , Inmunoglobulina G/sangre , Enfermedades Orbitales/inmunología , Enfermedades Orbitales/patología , Enfermedades Orbitales/diagnóstico , Femenino , Masculino , Persona de Mediana Edad , Xantomatosis/inmunología , Xantomatosis/patología , Adulto , Granuloma/inmunología , Granuloma/patologíaRESUMEN
A recent study described a rare subtype of tuberous sclerosis complex ( TSC )-mutated renal cell carcinoma primarily characterized by Xanthomatous giant cell morphology. Only 2 cases in young individuals have been reported so far, making the correct diagnosis challenging from a pathological perspective. It remains unknown whether this tumor represents an independent subtype or belongs to other TSC -mutated tumors. We conducted a clinicopathologic evaluation and immunohistochemical profiling of 5 cases of Xanthomatous Giant Cell Renal Cell Carcinoma (XGC RCC) with confirmed TSC2 mutations through targeted DNA sequencing. In addition, we analyzed transcriptomic profiles using RNA-seq for the following samples: XGC RCC, Low-grade Oncocytic tumors (LOT), High-grade Oncocytic tumors/Eosinophilic Vacuolar Tumors (HOT/EVT), Eosinophilic Solid and Cystic Renal Cell Carcinomas (ESC RCC), Chromophobe cell Renal Cell Carcinomas (ChRCC), Renal Oncocytomas (RO), clear cell Renal Cell Carcinomas (ccRCC), and normal renal tissues. There were 2 female and 3 male patients, aged 22 to 58 years, who underwent radical nephrectomy for tumor removal. The tumor sizes ranged from 4.7 to 9.5 cm in diameter. These tumors exhibited ill-defined boundaries, showed an expansive growth pattern, and featured distinctive tumor giant cells with abundant eosinophilic to Xanthomatous cytoplasm and prominent nucleoli. All tumors had low Ki-67 proliferation indices (<1%) and demonstrated immune reactivity for CD10, PAX8, CK20, CathepsinK, and GPNMB. Next-generation sequencing confirmed TSC2 mutations in all cases. RNA sequencing-based clustering indicated a close similarity between the tumor and ESC RCC. One patient (1/5) died of an accident 63 months later, while the remaining patients (4/5) were alive without tumor recurrences or metastases at the time of analysis, with a mean follow-up duration of 43.4 months. Our research supports the concept that Xanthomatous giant cell renal cell carcinoma (XGC RCC) shares clinicopathological and molecular characteristics with ESC RCC and shows a relatively positive prognosis, providing further support for a close morphologic spectrum between the two. We propose considering XGC RCC as a distinct subtype of ESC RCC.