IgG4-related Sclerosing Disease Research Articles

Gall Bladder Mass-always a Cancer? Think Outside the Box!

Background: Gallbladder cancer (GBC) is endemic in northern part of India . Any patient presenting with a gallbladder mass is considered malignant until proved otherwise. Many benign conditions of gallbladder mimic GBC and immunoglobulin G4(IgG4) disease is amongst the rare ones. IgG4 related sclerosing disease is a systemic inflammatory disease with multiorgan involvement. It is attributed to an interplay of autoimmune, genetic and molecular pathways. It is rare and presentation is atypical. Disease can be localized or diffuse type. Diffuse type usually associated with IgG4 sclerosing cholangitis or pancreatitis while localized type of IgG4 cholecystitis can imitate maliganancy. Hence a high index of suspicion is required when the presentation is atypical. Case report: Our patient, a middle aged lady presented with vague right upper abdominal pain with no associated symptoms. On evaluation she was found to have mass lesion in fundus of gallbladder while her liver function, tumour markers (CEA and CA 19-9) and serum IgG4 level was normal. Computed tomography and magnetic resonance imagining was suggestive of a malignant lesion. She underwent anticipatory extended cholecystectomy in view of the same(Fig 1),but final histopathology revealed chronic sclerosing cholecystitis with lymphocytic infilrate and storiform fibrosis, hence IgG4 immunohistochemistry was performed and was positive. Conclusion: IgG4 cholecystitis is exceptionally rare and a difficult diagnosis to make in the pre-operative phase especially since serum IgG4 was normal. Due to uncertanity of diagnosis and aggressive nature of GBC ,timely surgery is prudent and deligent histopathological analysis of resected specimen is pivotal.

PEGylated Liposomal Methyl Prednisolone Succinate does not Induce Infusion Reactions in Patients: A Correlation Between in Vitro Immunological and in Vivo Clinical Studies.

PEGylated nanomedicines are known to induce infusion reactions (IRs) that in some cases can be life-threatening. Herein, we report a case study in which a patient with rare mediastinal and intracardiac IgG4-related sclerosing disease received 8 treatments of intravenously administered PEGylated liposomal methylprednisolone-succinate (NSSL-MPS). Due to the ethical requirements to reduce IRs, the patient received a cocktail of premedication including low dose of steroids, acetaminophen and H2 blockers before each infusion. The treatment was well-tolerated in that IRs, complement activation, anti-PEG antibodies and accelerated blood clearance of the PEGylated drug were not detected. Prior to the clinical study, an in vitro panel of assays utilizing blood of healthy donors was used to determine the potential of a PEGylated drug to activate complement system, elicit pro-inflammatory cytokines, damage erythrocytes and affect various components of the blood coagulation system. The overall findings of the in vitro panel were negative and correlated with the results observed in the clinical phase.

Pulmonary Hyalinizing Granuloma With Elevated IgG4 Levels, A Link To IgG4-Related Sclerosing Disease?

Pulmonary Hyalinizing granuloma (PHG) is a rare form of lung pathology, often initially presenting as nodules prompting workup for possible malignancy. We present a case of a 29-year-old man with an irregularly shaped, growing hilar mass, suggested to be PHG on initial biopsy, later confirmed by surgical pathology examination of the entire lung after pneumonectomy. The pathology findings also reported many of the histopathologic features found in IgG4-related sclerosing disease (ISD). This gross presentation and the correlation with ISD may have therapeutic implications in the management of PHG.

Pulmonary Nodular Lymphoid Hyperplasia.

Pulmonary nodular lymphoid hyperplasia is an uncommon reactive lymphoproliferative disorder that presents as an asymptomatic lung mass. The histopathologic diagnosis of pulmonary nodular lymphoid hyperplasia may be challenging because of its morphologic overlap with other diseases, such as extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and immunoglobulin G4-related sclerosing disease. Despite the similarities, there are distinctive morphologic and phenotypic features that allow for the correct diagnosis in the majority of cases. This review aims to discuss the clinicopathologic features of pulmonary nodular lymphoid hyperplasia and contrast them with its histopathologic mimickers.

A Curious Case of Autoimmune Pancreatitis with IgG4 Related Sclerosing Cholangitis

IgG4-related sclerosing cholangitis (IgG4-SC) is an uncommon condition recently recognized as an independent disease entity in hope to better specify future diagnosis modalities and treatment. Its exact pathophysiologic mechanism has yet to be identified, but evidence of submucosal fibrosis and inflammatory cells infiltrating biliary ducts have been noted on biopsies. We present a case of IgG4 related ascending cholangitis in a patient with obstructive jaundice secondary to autoimmune pancreatitis. Patient is a 54 year-old male admitted to the hospital in July 2016 with symptoms of obstructive jaundice. MRI abdomen revealed possible mass on pancreas with a sausage-like appearance of the organ. LFTs were elevated, CEA was 652.9, and IgG4 was 464. Liver biopsy was consistent with IgG4 autoimmune pancreatitis. The patient responded well to steroids and the CEA levels dropped. He followed up in office and steroids were tapered in February 2017 with plans to start Azathioprine. In December 2017 he went to the hospital for obstructive jaundice and RUQ pain. IgG4 was >700. MRI showed relapse of AIP with cystic changes at the level of the pancreatic neck as well as a 1 cm long stricture of the proximal intrapancreatic portion of the CBD and wall thickening of the common hepatic duct and CBD, findings indicative of sclerosing cholangitis or IgG4 related sclerosing disease. The patient was again started on steroids and is currently doing well. IgG4-SC is a novel entity which has been documented to be associated with conditions such as autoimmune pancreatitis, primary sclerosing cholangitis, and cholangiocarcinoma. There is increased prevalence in men, the elderly, and patients with multiorgan involvement. Several factors have been used for diagnosis, such as a serum IgG4 levels, cholangiography, and IgG4-positive plasma cell infiltration on biopsy. Despite the fact that little is known regarding treatment options, IgG4-SC has been documented to respond to steroids. The precise dose of steroids to be utilized has yet to be established. Alternative treatment modalities consist of biliary stent placement, drainage, or surgical intervention. To date, several diagnostic criteria have been proposed as means to help identify IgG4-SC, and other related diseases. It is evident that further comparative diagnostic and treatment studies need to be conducted, as treatment remains inconsistent due to a lack of standardized criteria.

Xanthogranulomatous cholecystitis: pseudo tumor of gall bladder

Introduction: Xanthogranulomatous cholecystitis is an uncommon inflammatory disease of gall bladder with reported prevalence of 0.7% in US and 10% in India and Japan. Its final diagnosis can be made only after malignancy is ruled out on histopathological examination of resected gall bladder. Case presentation: 60 years female presented with complaints of pain in right upper abdomen, loss of appetite for 3 months. Physical examination was normal. Abdominal examination revealed tenderness in right upper quadrant and a hard, non tender globular mass suggestive of gall bladder lumpBlood parameters were normal. Contrast enhanced CT scan abdomen reported grossly distended gall bladder with 12 mm calculus at neck, pericholecystic fat stranding, asymmetric wall thickening and few lymph nodes. Serum CA19.9 -103 IU/l.. Diagnostic laparoscopy that ruled out intraperitoneal disease followed by cholecystectomy and wedge resection of liver. Intraoperatively, gall bladder was found to be hard, elongated, densely adhered to GB fossa with multiple calculi in it. Histopathologically greyish-yellow streaks in the gallbladder wall, transmural inflammation with dense lymphoplasmacytic infiltration and fibrosis, perineural plasma infiltrate, one of the areas showing storiform fibrosis, suggesting xanthogranulomatous or IgG4 cholecystitis. No malignancy present. Serum IgG levels were raised but IgG4 levels were normal. Immunohistochemistry for IgG4 plasma cells in the specimen was negative. Conclusion: Xanthogranulomatous cholecystitis is a perfect mimicker of carcinoma gall bladder and diagnosis is difficult, both pre and intraoperatively. It is a differential for IgG4 cholecystitis also which though rare isolated entity, is usually a part of spectrum of IgG4 related sclerosing diseases.

DIAGNOSIS AND TREATMENT OF AUTOIMMUNE PANCREATITIS. (REVIEW)

The autoimmune pancreatitis was described in 1961 as “a primary inflammatory sclerosis of the pancreas”. Subsequent reports have described this disease as lymphoplasmacytic sclerosing pancreatitis, chronic sclerosing pancreatitis, nonalcoholic duct-destructive pancreatitis, inflammatory pseudotumor of pancreas, etc. The concept of autoimmune pancreatitis was proposed by Yoshida et al. in 1995. Numerous clinical, serological, radiological and pathological studies were conducted. Diagnostic criteria, serological markers and pathological features were introduced to facilitate autoimmune pancreatitis patients management. Also data were obtained indicating autoimmune pancreatitis as a part of new clinicopathological entity - IgG4-related sclerosing disease. Important issue is surgical treatment of autoimmune pancreatitis which is performed in case of suspected pancreatic cancer as a rule. Thus, autoimmune pancreatitis is an actual problem of modern medicine and requires further study.

Clinicopathologic analysis of extranodal Rosai-Dorfman disease of breast: a report of 12 cases

To investigate the clinicopathologic features, diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease(RDD)of the breast. Twelve cases of extranodal RDD of the breast were analyzed using hematoxylin-eosin stain and immunohistochemical staining.The morphological features and immunophenotype were observed by light microscopy, accompanied with a review of the literature. Twelve cases of extranodal RDD of the breast are presented. The patients were women and their ages ranged from 15 to 55 years (mean =37 years). Except one patient with involvement of the right breast and ipsilateral axillary lymph nodes, other eleven patients' disease confined to the breast. Seven cases located in the right breast, the other five cases in the left breast. Microscopically, it showed a typical morphology, characterized by diffuse infiltration of large histiocytes, lymphocytes and plasmocytes that formed irregular nodular structure with light and dark appearance under microscope. Spindling of histiocytosis often arranged in a vague storiform with prominent fibrosis, accumulation of foamy histiocytes and scattered atypical nuclei. The characteristic histocytes and emperipolesis could not be seen obviously in extranodal RDD of the breast.Immunohistochemical staining showed histocytes were strongly positive for S-100 protein, CD68 and CD163, while negative for CD1a. Extranodal RDD of the breast is very rare. Because of unconspicuous emperipolesis and lack of specificity in clinic and images, extranodal RDD of the breast is easy to be misdiagnosed as other histiocytosis. The microscopic differential diagnosis includes idiopathic granulomatous mastitis, infective granulomas, langerhans cell histiocytosis, IgG4 related sclerosing disease, Erdheim-Chester disease, benign fibrous histiocytoma, malignant fibrous histiocytoma and Hodgkin Lymphoma. Light and dark appearance under microscope is the important clue in diagnosis of extranodal RDD in breast. Immunohistochemistry is also helpful.

Management of IgG4-related Sclerosing Disease

IgG4-related sclerosing disease is a systemic syndrome that is distinguished by high levels of both IgG4 and IgG4-positive lymphoplasmocytic infiltrative lesions in the body. We report an occurrence of orbital IgG4-related disease in a 62-year-old Hispanic male. IgG4 related sclerosing disease involving the orbit affects patients ranged from ages 30 to 86 with a 1:1 male to female ratio (1,2). The patient presented with bilateral painless, progressive nodular lesions of the lacrimal glands. Pathologic evaluation from the biopsy of the orbital mass showed an increase in IgG4 positive plasma cells, fibrosclerosis, and lymphoid hyperplasia. The patient was treated with a high dose of oral prednisone for a period of 3 months and was then placed on a maintenance dose with rapid resolution of both orbital masses. The work-up, diagnostic tools, and treatment of IgG4 disease are discussed. It is important to note that IgG4 can affect all orbital structures and physical findings, lab results, and biopsy results are required for appropriate diagnosis and management.

Unmasking IgG4-related autoimmune pancreatitis from pancreatic cancer: A lesson learned

Introduction: Autoimmune pancreatitis (AIP) is difficult to distinguish from pancreatic cancer. An AIP has a similar clinical presentation and is one component of a systemic disease, Immunoglobulin G4 (IgG4) related sclerosing disease. IgG4 related sclerosing disease is characterized by extensive IgG4 positive plasma cells and t-lymphocytes that may involve the pancreas and other organs. case report: A 77-year-old male presented with nausea, vomiting, decreased appetite, and 39 lbs weight loss over three months. An endoscopic ultrasound showed one 3.3x2.7 cm mass on the tail of the pancreas extending towards the splenic hilum with no lymph node involvement. A fine needle aspiration was indeterminate. the patient underwent distal pancreatectomy and splenectomy for presumed pancreatic cancer, however, the pathology report was negative for malignancy. Instead, the microscopic examination revealed dense lymphoplasmacytic infiltrate staining positive for IgG4, onion-skin pattern fibrosis, and lobular atrophy in the medium sized pancreatic duct that was consistent with AIP. conclusion: IgG4 related sclerosing

Immunoglobulin G4-related sclerosing disease of the paranasal sinuses: A case report and literature review.

Objective:Immunoglobulin G4 (IgG4) related sclerosing disease (RSD) of the paranasal sinuses is a rare lesion of dense lymphoplasmacytic tissue, with a high proportion of IgG4+ plasma cells. We presented a rare case of IgG4-RSD with isolated involvement of the paranasal sinuses in the absence of multiorgan involvement.Methods:A case report and comprehensive literature review.Results:To our knowledge, only 11 cases of IgG4-RSD with paranasal sinus involvement have been reported. Patients with IgG4-RSD commonly present with epistaxis and symptoms that mimic chronic rhinosinusitis, e.g., rhinorrhea, nasal obstruction, and facial pressure. On imaging, an expansive and erosive process is described. Surgery provides tissue for immunohistologic evaluation; however, there is a paucity of evidence about the direct extent of surgical resection or medical therapies. Postoperative steroids were typically started, although the regimen was not standardized.Conclusion:Few cases of paranasal sinus IgG4-RSD have been reported in the literature. Evidence-based recommendations regarding treatment and surveillance of paranasal sinus IgG4-RSD are lacking; however, most reports describe systemic steroids as the mainstay of treatment. This single subject analysis, with a review of previously reported cases adds to the expanding body of data related to this rare disorder.

Management of IgG4-related sclerosing disease

IgG4-related sclerosing disease is a systemic syndrome that is distinguished by high levels of both IgG4 and IgG4-positive lymphoplasmocytic infiltrative lesions in the body. We report an occurrence of orbital IgG4-related disease in a 62-year-old Hispanic male. IgG4 related sclerosing disease involving the orbit affects patients ranged from ages 30 to 86 with a 1:1 male to female ratio (1,2). The patient presented with bilateral painless, progressive nodular lesions of the lacrimal glands. Pathologic evaluation from the biopsy of the orbital mass showed an increase in IgG4 positive plasma cells, fibrosclerosis, and lymphoid hyperplasia. The patient was treated with a high dose of oral prednisone for a period of 3 months and was then placed on a maintenance dose with rapid resolution of both orbital masses. The work-up, diagnostic tools, and treatment of IgG4 disease are discussed. It is important to note that IgG4 can affect all orbital structures and physical findings, lab results, and biopsy results are required for appropriate diagnosis and management.

Retroperitoneal IgG4-related sclerosing disease mimics renal pelvic cancer on 18F-FDG PET/CT

Retroperitoneal IgG4-related sclerosing disease mimics renal pelvic cancer on 18F-FDG PET/CT

The importance of IgG4 in the predictive model of thyroiditis.

SummaryImmunoglobulin (Ig)G4-related sclerosing disease (IgG4-RSD) is a new disease entity first proposed with regard to autoimmune pancreatitis. A 67-year-old male patient was examined because of weight loss and an abdominal pain. Based on the clinical characteristics, laboratory parameters and ultrasound features, we identified the diagnosis of the IgG4-related systemic disease (IgG4-RSD), that was confirmed by the histopathological analysis after the biopsy of the head of pancreas. After confirmation, we started with the corticosteroid therapy with a good clinical, biochemical and morphological response. During the previous therapy, the disturbance of glucoregulation appeared, so we had to change the modality of treatment. We decided to add Azathioprine to the therapy in a dose of 150 mg/day. We achieved a stable phase of the disease with IgG 4.37 g/l and IgG4 0.179 g/l, and with no side effects from the therapy.Learning points There are potential clinical applications of identifying subsets of patients with IgG4 thyroiditis (FVHT and Riedel thyroiditis).A trial of immunosuppressive therapy should be included if a resection is deemed inadvisable.In particular, cases of FVHT that mimic malignancy, tissue and serum IgG4 may provide supportive diagnostic information.

Inflammatory pseudotumor of eyelid: a probable IgG4-related sclerosing disease clinically mimicking eyelid pilomatrixoma

BackgroundOcular adnexal IgG4-related sclerosing disease (IgG4-SD) has been categorized as a novel disease entity. It is characterized by stromal sclerosis and an infiltration of mass-forming lymphoplasmic cells containing many IgG4-positive plasma cells. Although ocular adnexal tissue involvement has been increasingly reported, a focal nodular sub-brow mass is not typical in an IgG4-SD presentation. We report a rare case of probable ocular adnexal IgG4-SD that clinically mimicked eyelid pilomatrixoma.Case presentationA 42-year-old woman presented with a nodular mass in her left sub-brow area. The initial clinical impression of her lesion was eyelid pilomatrixoma. However, the final pathologic diagnosis was IgG4–SD, but extranodal marginal zone B-cell lymphoma could not be excluded. The patient underwent testing to determine tumor malignancy and systemic IgG4-SD involvement. Laboratory testing showed normal IgG and IgG4 serum levels and imaging revealed no remarkable findings. Oral prednisolone was administered and slowly tapered to manage the possible remnant lesion and to prevent disease recurrence. Two years after initiating therapy, there was no evidence of relapse. The patient is under close surveillance for signs of recurrence, systemic involvement, and potential malignant transformation.ConclusionsWe found an unusual case of probable ocular adnexal IgG4-SD, which presented as a unilateral restricted mass involving the sub-brow area. Although the mass was surgically removed, systemic steroid treatment and long-term surveillance were initiated due to the possibility of recurrence, the potential association with systemic disease, and the potential development of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma.

Inflammatory Fibroid Polyps of Gastrointestinal Tract Rarely Show Increased IgG4 Expression

Inflammatory Fibroid Polyp (IFP) of gastrointestinal tract is characterized by concentric perivascular fibrosis and a mixed inflammatory infiltrate rich in eosinophils and also few plasma cells. IgG4-related sclerosing diseases (IgG4-RSD) are a heterogenous group of disorders described in many organs, characterized by a significant increased of IgG4+ plasma cells in a context of storiform fibrosis, obliterative vasculitis and mixed inflammatory infiltrate containing eosinophils. The histological similarities between IFP and IgG4-RSD prompted the present study in the attempt to find a possible link between IgG4 over-expression and IFP. The expression of IgG4 and IgG was evaluated in a series of 23 cases of IFP belonging to 23 patients. All cases were reviewed by two pathologists and the histological diagnosis confirmed. Immunohistochemistry for antibodies anti-IgG, anti-IgG4, and anti-IgA was performed on all cases and the results were evaluated by two observers. One case of IFP out of 23 (4.3%) fulfilled the IgG4-RSD histological criteria. The case did not differ histologically from the others. The patient did not present either a raised serological level of IgG4, nor other sign of IgG4-RSD. IgG4 increased expression can be rarely observed in IFP. Steroid therapy in cases of IFP with abundant IgG4+ plasma cells, especially in patients with multiple tumors, could be considered as an alternative to surgical treatment.

A Rare and Emerging Entity: Sinonasal IgG4–related Sclerosing Disease

Background:Immunoglobulin G4 (IgG4) related sclerosing disease (rSD) is a new disease entity, first described in 2001, that involves autoimmune pancreatitis. Considered a systemic disease with lesions described in multiple organ systems, IgG4-rSD that affects the sinonasal region is rare. Our goal was to highlight the sinonasal presentation of this unique disease and to review previously reported adult cases from 2003 to 2014.Methods:Case report (a 72-year-old man who presented with left exophthalmos, periorbital pain, and epiphora) and review of the literature.Results:Radiographic workup with computed tomography and magnetic resonance imaging demonstrated a left sinonasal mass that involved the left maxillary and ethmoid sinuses, with surrounding bony destruction and orbital invasion. Nasal endoscopy demonstrated a fibrous lesion emanating in the middle meatus, with surrounding mucosal inflammation. The patient underwent an endoscopic biopsy, medial maxillectomy, and ethmoidectomy with tumor debulking. Pathology demonstrated inflamed respiratory mucosa with dense lymphoplasmacytic infiltrate and fibrosis; flow cytometry demonstrated no malignant cell populations; immunophenotyping demonstrated multiple foci of IgG4 cells. Plasma IgG4 was elevated in the setting of normal total IgG. The patient was treated with postoperative systemic and topical corticosteroids. Surveillance imaging studies and nasal endoscopy demonstrated disease resolution without recurrence.Conclusions:Sinonasal IgG4-rSD is a rare disease that can present with bony and soft-tissue invasion. This was an exceptional case, with osseous involvement and orbital invasion. Immunohistologic workup is essential for diagnosis. It is important to differentiate this disease from sinonasal tumors. Treatment includes corticosteroids and surgical debulking. Sinonasal IgG4-rSD represents an emerging disease that may present challenges for future rhinologists.

Immunoglobulin G4-Related Kidney Disease: A Comprehensive Pictorial Review of the Imaging Spectrum, Mimickers, and Clinicopathological Characteristics.

Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

Plasma Cell (Zoon) Balanitis

Numerous IgG4 plasma cells and a high IgG4/IgG plasma cell ratio are important criteria in the diagnosis of IgG4-related sclerosing disease (IgG4-RSD), a steroid-responsive fibroinflammatory disorder. There is also a growing list of other inflammatory disorders that may mimic IgG4-RSD with many IgG4 plasma cells; however, limited data exist concerning whether plasma cell (Zoon) balanitis (PCB) is among these disorders. We, therefore, reviewed the clinical, histologic, and immunohistochemical features of PCB in 17 patients, including evaluation of CD3, CD20, CD138, κ, λ, IgG, IgG4, IgM, IgA, and IgD. All biopsies showed an infiltrate rich in plasma cells with variable numbers of B and T cells. IgG4 counts in the areas with the greatest density varied from 1/HPF to >200/HPF. Six of 17 (35.3%) cases demonstrated 50 or more IgG4 plasma cells/HPF, with an IgG4/IgG ratio of >40%, at least focally, in 2 of these cases. The plasma cells were clearly polytypic in 12/15 evaluable cases, with an increased proportion of κ-positive plasma cells in 3 (≥4 to 5:1). None of the patients had other clinical evidence of IgG4-RSD or a lymphoproliferative disorder. In conclusion, this study demonstrates that PCB should be added to the growing list of inflammatory disorders that can have significantly increased IgG4 plasma cells but which do not represent IgG4-RSD and which can have increased κ-positive plasma cells in the absence of malignant lymphoma.

Renal cell carcinoma mimicking IgG4-related pseudotumor in autoimmune pancreatitis.

Autoimmune pancreatitis is classified into two distinct clinical profiles. Type 1 autoimmune pancreatitis (AIP) is considered to be a manifestation of a novel clinicopathological entity called IgG4 related sclerosing disease, diagnosed using the Mayo Clinic HISORt criteria. Extra-pancreatic manifestations can include involvement of bile ducts, salivary gland, lung nodules, thyroiditis, tubulointerstitial nephritis, renal masses, and retroperitoneal fibrosis. Type 2 autoimmune pancreatitis on the other hand is confirmed by histologically seen duct centric pancreatitis without elevation of IgG4 or involvement of other organs. In type 1 autoimmune pancreatitis, extrapancreatic manifestations like bile duct strictures, tubulointerstitial nephritis, renal nodules, retroperitoneal fibrosis respond to steroid therapy. We present a case of type 1 autoimmune pancreatitis in which the renal mass did not respond to steroid therapy and was later on found to be renal cell carcinoma. To the best of our knowledge this is only the third reported case of autoimmune pancreatitis in which the patient had renal cell carcinoma. Our case highlights the importance of close follow up of lesions that do not respond to steroid treatment which in this case proved to be renal cell cancer.