Non-necrotizing Granulomatous Inflammation Research Articles

Unexpected Presentations of Histiocytic Disorders as Mass Lesions in the Central Nervous System and Spine

Abstract Introduction/Objective A number of neoplastic and non-neoplastic hematolymphoid lesions can involve the CNS and spine. The majority are lymphomas, but some are rare lesions that mimic primary neoplasms clinically, radiologically, and even pathologically. Here, we present our experience of such cases. Methods/Case Report Pathology archives were searched, and five cases representing different challenges were identified to review clinical, radiological, and pathologic features. Case 1. 31-year-old female presented with vision changes and hearing loss and numerous supratentorial extra-axial masses, suspicious for meningiomatosis. Extensive lymphoplasmacytic infiltrate was seen at Intraoperative consult (IOC). Final diagnosis was Rosai-Dorfman disease with features of IgG4 related disease. Case 2. 31-year-old female with Chronic myeloid leukemia (CML), which progressed to AML presented with a right frontal brain mass. IOC displayed atypical hematolymphoid infiltrate. Final diagnosis was myeloid sarcoma. Case 3. 29-year-old male presented with a T10 vertebral lesion. IOC showed hypercellular tissue with atypia and was deferred. Subsequently, histiocytic sarcoma was diagnosed. Case 4. 22-year- old male presented with a T3-T4 intradural, extramedullary mass radiologically thought to be a meningioma. No IOC was requested. Final diagnosis was juvenile xanthogranuloma. Case 5. 36-year-old male presented with a C7-T1 dura-based mass. IOC revealed granulomatous inflammation, eventually resulting in a diagnosis of non-necrotizing granulomatous inflammation, suggesting sarcoidosis. Results (if a Case Study enter NA) NA Conclusion These cases highlight the importance of incorporating all clinical and radiological findings into pathologic evaluation, while being alert for mimickers and pitfalls. Both practicing general surgical pathologists and neuropathologists should remain alert to such unexpected and unusual cases especially at the time of IOC and initiate appropriate work-up with expedited diagnostic protocols such as intraoperative cultures, flow cytometry, and cytogenetics, as the differential diagnosis is broad with inflammatory, infectious, and neoplastic possibilities, presenting a variety of challenges.

Imaging findings in a rare case of alveolar, hepatic and splenic sarcoidosis presenting with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is an acute haematological disorder characterized by severe ADAMTS13 enzyme deficiency, leading to consumptive thrombocytopenia, mechanical haemolysis, and organ damage. Its association with multisystemic sarcoidosis is extremely rare and, to the best of our knowledge, described in only two cases so far. We present the case of a 52-year-old woman with TTP and antibodies anti-ADAMTS13, showing computed tomography (CT) and magnetic resonance imaging (MRI) evidence of pulmonary, hepatic, and splenic lesions initially reported as ischemic/inflammatory changes. A follow-up MRI of the abdomen revealed increased evidence of the liver lesions, focal intrahepatic bile duct dilatation, splenic lesions, and enlarged hepatic hilar nodes. The follow-up chest CT showed increased evidence of the parenchymal lung consolidations. Given the radiological persistence of those alterations and the history of TTP, the hypothesis of an IgG4-related disease was then made. The IgG4 levels were found to be normal, while the histological examination of the liver revealed non-necrotizing granulomatous chronic inflammation. Elevated levels of angiotensin-converting enzyme were found, and the QuantiFERON-TB Gold test was negative for tuberculosis infection. Thus, the overall clinical picture was consistent with multisystemic sarcoidosis (alveolar, hepatic, and splenic). The diagnosis of sarcoidosis, already challenging due to the variability of its clinical presentation, can become even more complicated when it manifests with uncommon haematological manifestations such as TTP, along with non-specific extra-pulmonary involvement. While imaging aids in documenting organ damage, the definitive diagnosis of sarcoidosis necessitates histologic confirmation of noncaseating granulomas and the exclusion of other possible granulomatous diseases.

Phenotypes and Serum Biomarkers in Sarcoidosis.

Sarcoidosis is a multisystem disease, which is diagnosed on a compatible clinical presentation, non-necrotizing granulomatous inflammation in one or more tissue samples, and exclusion of alternative causes of granulomatous disease. Considering its heterogeneity, numerous aspects of the disease remain to be elucidated. In this context, the identification and integration of biomarkers may hold significance in clinical practice, aiding in appropriate selection of patients for targeted clinical trials. This work aims to discuss and analyze how validated biomarkers are currently integrated in disease category definitions. Future studies are mandatory to unravel the diverse contributions of genetics, socioeconomic status, environmental exposures, and other sociodemographic variables to disease severity and phenotypic presentation. Furthermore, the implementation of transcriptomics, multidisciplinary approaches, and consideration of patients' perspectives, reporting innovative insights, could be pivotal for a better understanding of disease pathogenesis and the optimization of clinical assistance.

1234 Enlarging Pericardial Cyst Compressing the Right Ventricle

Abstract 52-year-old Indian builder initially presented to his General Practitioner with exertional breathlessness but no angina. His medical history included previous tuberculosis infection, Type 2 Diabetes Mellitus and hypothyroidism. He was referred to the cardiologist to exclude coronary artery disease. Stress testing found T-wave inversion in the inferior electrocardiogram leads and transthoracic echocardiogram showed reduced right ventricular (RV) function with basal wall hypokinesia. Cardiac Magnetic Resonance Imaging (MRI) revealed a 5cm x 2.5cm pericardial cyst (Figure 1a). As imaging suggested minimal RV compression and no venous obstruction, the patient was investigated for other causes of breathlessness. Repeat computed tomography (CT) two years later demonstrated enlargement of the cyst (6cm x 3.6cm) with contrast reflux into the inferior vena cava (IVC) (Figure 1b). Due to worsening breathlessness, surgical excision was offered. Surgery was attempted through a lower hemi-sternotomy. However, due to limited access to the cyst and thickened pericardium, median sternotomy was performed. The cyst measured 10cm x 7cm and was heavily adherent to the RV wall, right atrium and IVC preventing complete excision. The cyst was therefore unroofed, releasing cheese-like material suspicious of a tuberculous cyst (Figure 2). Adhesiolysis was undertaken to remove remaining cyst wall. Prior to closure, inflamed pericardial tissue was excised. Histopathology revealed a chronically inflamed pseudocyst with necrotising and non-necrotising granulomatous inflammation. Ziehl-Neelsen, Grocott, Gram and periodic acid-Schiff-diastase stains were negative. Intraoperative and histological findings and history of tuberculosis suggested a diagnosis of tuberculous pericardial cyst. The patient recovered uneventfully with significant symptomatic improvement at 2-month follow-up.

Treatment of Orofacial Granulomatosis—9-Month Follow-up: A Case Report

Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher's granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema.

НОВІ ПІДХОДИ ДО ВЕДЕННЯ ХВОРИХ НА САРКОЇДОЗ ЛЕГЕНЬ (методичний посібник)

Sarcoidosis is an inflammatory multi-systemic disease of unknown origin with wide spectrum of clinical manifestations. Sarcoidosis may affect practically any organ — predominantly lungs, lymphatic system, skin, eyes alone or in combination. The disease is characterized by formation of non-caseous granuloma. In 1999 American Thoracic society (ATS), European Respiratory society (ERS) and World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) adopted an international consensus statement on diagnosis and treatment of sarcoidosis «Statement on Sarcoidosis». Based on ATS, ERS and WASOG 1999 Statement the national documents such as “Evidence-based adapted clinical guideline on sarcoidosis” and “Unified clinical protocol of primary, secondary (specialized) and tertiary (highly-specialized) medical care for sarcoidosis” approved by MOH of Ukraine (decree # 634 dated 08 Sep 2014) were developed by current authors in Ukraine. For a more detailed description of the principles of diagnosis and treatment of pulmonary sarcoidosis, we have developed and published methodological guidelines "Diagnosis of respiratory tract sarcoidosis" (2014) and "Treatment of pulmonary sarcoidosis" (2018). In 2020 ATS experts published an updated «Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline», according to which the diagnosis of sarcoidosis was based on 3 criteria: compatible clinical symptoms, presence of non-necrotizing granulomatous inflammation in one or more tissue samples (not always required) and exclusion of alternative causes of granulomatous disorder. Finally, in 2021 ERS task force report was published “ERS clinical practice guidelines on treatment of sarcoidosis”, which outlined completely new approaches to management of sarcoidosis patients. According to this document, a decision about treatment of patient depends on 2 major factors: risk of death or organ failure and deterioration of quality of life of a patient. In addition, main principle of management of sarcoidosis patients is a balance between: a) minimization of risk of disability or death due to lung injury and decrease of quality of life, and b) risk of comorbidity and reduction of quality of life due to corticosteroid or other therapies. Methodical guideline focuses on analysis of major statements of these documents, disputable questions of initial therapy choice in patients with pulmonary sarcoidosis.

Bilateral eyelid swelling as the presenting sign of subcutaneous sarcoidosis.

An 18-year-old Black female presented with a 2-year history of bilateral upper eyelid swelling and the recent onset of multiple subcutaneous nodules on the arms. She had previously undergone evaluation and treatment for presumed angioedema. Biopsies of the eyelid and an arm nodule demonstrated non-necrotizing granulomatous inflammation with special stains negative for acid-fast bacilli and fungi, and the patient was diagnosed with subcutaneous sarcoidosis. The isolated finding of bilateral eyelid swelling 2 years prior to the onset of additional cutaneous findings led to a significant delay in diagnosis, highlighting the importance of considering sarcoidosis in the differential diagnosis for bilateral eyelid swelling.

Lady Windermere Syndrome: Historical Perspective and Report of a Rare Case

Abstract Introduction/Objective Lady Windermere syndrome refers to Mycobacterium avium complex (MAC) pulmonary disease with an isolated lingular or middle lobe pattern of involvement. This is thought to be due to habitual suppression of the cough voluntarily. Typical demographics are that of elderly female patient. Although the etiology remains unclear high prevalence of pectus excavatum, scoliosis and mitral valve prolapse has also been noted in these patients. The purpose of this report this to describe a case of this syndrome. Methods/Case Report A 64-year-old female with a history of breast carcinoma status post bilateral mastectomies, and thyroid papillary microcarcinoma, status post thyroidectomy, presented with hemoptysis. Clinically and radiological findings were consistent with pneumonia which improved but did not completely resolve following antibiotic treatment. Computerized tomography (CT) revealed progressive infiltrates and bronchoscopy cultures grew Mycobacterium avium complex (MAC). Repeat antibiotic therapy resulted in only marginal improvement. Repeat CT showed diffuse bronchiectatic changes with scattered nodularity and tree-in-bud opacities, with majority of involvement in the right middle lobe and lingula. Multiple interventions were employed to ameliorate the patient’s condition including a positive expiratory pressure device, gastroesophageal reflux disease (GERD) precautions and combinations of 2 drug therapies without significant improvement in her condition. It was ultimately decided that she may benefit from a right middle lobectomy. Histologic sections from the lobectomy specimen showed necrotizing and non-necrotizing granulomatous inflammation and bronchiolectasis. Rare acid-fast microorganisms were identified on AFB special satin. Lady Windermere syndrome was suggested in the diagnostic comment after expert consultation. Patient’s symptoms improved after surgery and additional samples show no growth of MAC. Results (if a Case Study enter NA) NA Conclusion Although rendering a definite diagnosis is challenging in such cases, the constellation of clinical, radiologic, and histologic finding can be used to raise the possibility of Lady Windermere syndrome.

Granulomatous myopathy: Sarcoidosis and beyond.

Non-necrotizing granulomatous inflammation is a rare but easily recognized histopathological finding in skeletal muscle biopsy. A limited number of diseases are known to be associated with non-necrotizing granulomatous myopathy. Once identified, a careful evaluation for evidence of extramuscular granulomatosis and other signs suggestive of sarcoidosis is warranted as about half of the patients have sarcoid myopathy. In addition, the presence of granulomatous myopathy should trigger a search for clinical and pathological clues of inclusion body myositis (IBM), which accounts for most of the remaining patients and can coexist with sarcoidosis. Recognizing the features of IBM in patients with granulomatous myopathy can potentially spare the patients from unnecessary exposure to immunosuppressive therapies. In patients whose granulomatous myopathy remain unexplained, further investigations should aim at identifying myasthenia gravis and other autoimmune disorders, especially those known to cause granulomatous inflammation in other organs. Laboratory investigations should include acetylcholine receptor, antimitochondrial, antineutrophil cytoplasmic, thyroglobulin, and thyroid peroxidase autoantibodies. In the appropriate clinical context, exposure to immune checkpoint inhibitors and chronic graft-vs-host disease can be causes of granulomatous myopathy. In cases of unexplained granulomatous myopathy, natural killer/T-cell lymphoma should be considered and careful histopathological examination for atypical cells and appropriate immunostaining is crucial. Identifying the etiology of granulomatous myopathy in each patient can guide appropriate treatment.

GRANULOMATOUS REACTION TO A MEDIASTINAL SEMINOMA

GRANULOMATOUS REACTION TO A MEDIASTINAL SEMINOMA

CAUGHT IN THE FRAY: NEUROSARCOIDOSIS PRESENTING AS CHRONIC RESPIRATORY FAILURE

CAUGHT IN THE FRAY: NEUROSARCOIDOSIS PRESENTING AS CHRONIC RESPIRATORY FAILURE

ATYPICAL PRESENTATION OF GRANULOMATOUS INFLAMMATION

ATYPICAL PRESENTATION OF GRANULOMATOUS INFLAMMATION

Robotic-assisted Bronchoscopy and Cone-beam CT: A Retrospective Series.

Robotic-assisted Bronchoscopy and Cone-beam CT: A Retrospective Series.

The risk of tuberculosis bacilli seeding during mediastinoscopy in mediastinal tuberculosis: Is it clinically important?

Mediastinoscopy is the gold standard for diagnosis in the absence of parenchymal lesions in mediastinal tuberculosis lymphadenitis. During mediastinoscopy biopsy, tuberculous bacillus seeding into the mediastinum is a rare complication. This study aimed to test the safety of mediastinoscopy in terms of Mycobacterium tuberculosis seeding in the mediastinum by microbiologically evaluating mediastinal lavage samples taken before and after biopsy. Classical cervical mediastinoscopy was performed all of patients and who were reported as granulomatous inflammatory events results of histopathological examinations and who underwent mediastinal lavage before and after biopsy, were included in the study. All the lavage fluids were tested for AFB and subjected to Mycobacterium tuberculosis PCR DNA testing and standard tuberculosis culture. The patients were divided into two groups, Group 1: Necrotizing granulomatous inflammation, Group 2: non-necrotizing granulomatous inflammation. The microbiological tests of the patients in Group 1 were negative before biopsy. However, in two patients of Group 1, the results of cultures of lavage fluids that taken from after biopsy were positive for tuberculosis. In all patients in Group 2, all microbiological tests of the lavage fluids were negative (Power of the decision: 99.8%, with 5% error). All of patients in group 1, Antituberculosis treatment was initiated and continued for 6 months. There weren’t seen any serius complications due to treatment and recurrence during the follow-up period. Mediastinoscopy can be used safely, with low morbidity and mortality rates and a high success rate for the diagnosis of mediastinal tuberculosis.

НОВІ ПІДХОДИ ДО ВЕДЕННЯ ХВОРИХ НА САРКОЇДОЗ ЛЕГЕНЬ

Sarcoidosis is an inflammatory multi-systemic disease of unknown origin with wide spectrum of clinical manifestations. Sarcoidosis may affect practically any organ — predominantly lungs, lymphatic system, skin, eyes alone or in combination. The disease is characterized by formation of non-caseous granuloma. In 1999 American Thoracic society (ATS), European Respiratory society (ERS) and World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) adopted an international consensus statement on diagnosis and treatment of sarcoidosis «Statement on Sarcoidosis». Based on ATS, ERS and WASOG 1999 Statement the national documents such as “Evidence-based adapted clinical guideline on sarcoidosis” and “Unified clinical protocol of primary, secondary (specialized) and tertiary (highly-specialized) medical care for sarcoidosis” approved by MOH of Ukraine (decree # 634 dated 08 Sep 2014) were developed by current authors in Ukraine. In 2020 ATS experts published an updated «Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline», according to which the diagnosis of sarcoidosis was based on 3 criteria: compatible clinical symptoms, presence of non-necrotizing granulomatous inflammation in one or more tissue samples (not always required) and exclusion of alternative causes of granulomatous disorder. Finally, in 2021 ERS task force report was published “ERS clinical practice guidelines on treatment of sarcoidosis”, which outlined completely new approaches to management of sarcoidosis patients. According to this document, a decision about treatment of patient depends on 2 major factors: risk of death or organ failure and deterioration of quality of life of a patient. In addition, main principle of management of sarcoidosis patients is a balance between: a) minimization of risk of disability or death due to lung injury and decrease of quality of life, and b) risk of comorbidity and reduction of quality of life due to corticosteroid or other therapies. Current review focuses on analysis of major statements of these documents, disputable questions of initial therapy choice in patients with pulmonary sarcoidosis. Key words: pulmonary sarcoidosis, diagnosis, treatment, glucocorticosteroids, immunosuppressants, cytokine inhibitors.

Clinical, Radiological and Histopathological Features of Patients With Lacrimal Gland Enlargement.

The purpose of this study was to describe the radiologic and histopathologic features of lacrimal gland in patients presenting with lacrimal gland enlargement. We retrospectively retrieved the data of patients with lacrimal gland enlargement in Farabi Eye Hospital between 2012 and 2017. These data included demographics, the patients' facial photographs, orbital CT-scans, and histopathological findings of lacrimal gland biopsies. Forty-seven patients (15 men and 32 women) were enrolled in this study with a median age of 37.9 years (range, 15-79 years). Histopathologic diagnoses were chronic dacryoadenitis in 26 cases (55.32%), IgG4-related disease in 6 patients (12.77%), two cases of acute dacryoadenitis, two cases of non-necrotizing granulomatous inflammation, two cases of Non-Hodgkin's B-cell lymphoma, two cases of adenoid cystic carcinoma and two cases of mixed tumor (4.26% each), as well as one case of conjunctival epithelial cyst, and one case of benign lymphoid tissue and fibrofatty tissue (2.13%). In two samples (4.26%), biopsy revealed normal lacrimal glands. Interestingly, in two cases with relapsing lacrimal gland enlargement, different histopathologic diagnoses were found in biopsies taken from each lacrimal gland at different times. The average size of enlarged lacrimal glands was 19.67 mm × 7.06 mm on axial CT scan and 19.44 mm × 6.20 mm on coronal CT scan. Tissue biopsy is needed for diagnosis of lacrimal gland enlargement because it is difficult to distinguish the type of the lacrimal gland pathology based solely on clinical or radiological presentation.

Abstract 17228: Not Just Another Knick In The Wall: An Unusual Case Of Multiple Arterial Aneurysms

Case Presentation: 47-year-old Liberian woman with a history of latent TB and no prior cardiac history presented with dyspnea, palpitations, and weight loss. She had fevers, tachycardia, and cervical lymphadenopathy. Cardiac exam showed widened pulse pressure, systolic and diastolic murmur, and features of heart failure. TTE showed dilated left ventricle with preserved ejection fraction, aortic root aneurysm compressing left atrium, severe aortic and mitral regurgitation, and moderate pericardial effusion with no tamponade. CT angiogram of neck, chest and abdomen showed right subclavian artery mycotic aneurysm, large left supraclavicular lymphadenopathy, multiple aortic arch, and descending thoracic aorta mycotic aneurysms. She underwent emergent surgical intervention. Intraoperative TEE revealed rupture of aortic root aneurysm into left ventricular outflow tract causing a fistula, perforated anterior mitral leaflet, and distortion of the left atrial wall. She underwent mitral and aortic tissue valve replacement, aortic root replacement, and a pericardial patch repair of the left atrial wall. Subsequently, she underwent right subclavian artery aneurysm resection, right carotid axillary bypass, and vertebral artery reimplantation. Aortic valve pathology was suggestive of endocarditis with negative cultures. Lymph node biopsy revealed non-necrotizing granulomatous inflammation with no evidence of acid-fast bacilli, fungi, and malignancy. Autoimmune workup was negative. A PET CT showed post-surgical inflammatory changes with no evidence of malignancy. Discussion: We describe an unusual case of multiple large arterial aneurysms causing severe valvular insufficiency requiring emergent surgical intervention. The patient underwent extensive workup which was unrevealing. She was treated for subacute bacterial endocarditis and suspected Bechet’s disease. Thus, the quest for a definitive diagnosis continues to elude us.

Risk of Cancer Among Sarcoidosis Patients With Biopsy-verified Nonnecrotizing Granulomatous Inflammation: Population-based Cohort Study.

To assess the long-term risk of hematologic cancers, invasive solid tumors, and nonmelanoma skin cancer (NMSC) among sarcoidosis patients with biopsy-verified nonnecrotizing granulomatous inflammation. We used Danish administrative registers with nationwide coverage to construct a cohort of 3892 patients with sarcoidosis and an age- and sex-matched comparison cohort of 38,920 population controls. For all patients, a biopsy demonstrating nonnecrotizing granulomatous inflammation had been obtained from the lower respiratory tract at the time of diagnosis. Study outcome was time to diagnosis of cancer. Follow-up began at time of sarcoidosis diagnosis and continued for up to 10 years. We calculated hazard ratios (HRs) as estimates of the cancer risk among the patients with sarcoidosis relative to that among the population controls and used cumulative incidence functions to calculate absolute 10-year risk estimates. We observed an increased long-term risk of hematologic cancers (HR during the first 2 years of follow-up: 2.71 [95% CI 1.18-6.25]; HR after > 2 years of follow-up: 2.12 [95% CI 1.29-3.47]) and NMSC (HR after > 2 years of follow-up: 1.82 [95% CI 1.43-2.32]) among the patients with sarcoidosis. An increased risk of invasive solid tumors was only observed during the first 2 years (HR 1.55, 95% CI 1.18-2.04). Compared with the population controls, the patients with sarcoidosis had an increased absolute 10-year risk of hematologic cancers (risk difference 0.56%, 95% CI 0.11-1.01%) and NMSC (risk difference 1.58%, 95% CI 0.70-2.47%). Sarcoidosis patients with biopsy-verified nonnecrotizing granulomatous inflammation have an increased long-term risk of hematologic cancers and NMSC compared with the general population.

PULMONARY SARCOIDOSIS WITH BONE INVOLVEMENT MIMICKING METASTATIC CANCER

Sarcoidosis is a disease of unknown etiology which can affect the lungs and lymph nodes, skin, eyes and other organs and, in rare cases, bones and bone marrow cells. We present a case of a 39-year-old male patient with an iliac bone involvement of sarcoidosis mimicking malignancy. Lymphoma and metastatic cancer proved a challenge to discard in differential diagnosis. He underwent multiple biopsies including tru-cut lung parenchyma and iliac bone biopsies. All of the biopsy specimens were reported as non-necrotising granulomatous inflammation. He underwent corticosteroid treatment. His response to the steroid treatment was insufficient so methotrexate was added to the corticosteroid therapy. Afterwards, clinical and radiological improvement was recorded. We aimed to highlight the utilization of taking new biopsies and histopathological re-evaluations in order to confirm the diagnosis and distinguish a benign disease from a malignant one.

PULMONARY SARCOIDOSIS WITH BONE INVOLVEMENT MIMICKING METASTATIC CANCER

Sarcoidosis is a disease of unknown etiology which can affect the lungs and lymph nodes, skin, eyes and other organs and, in rare cases, bones and bone marrow cells. We present a case of a 39-year-old male patient with an iliac bone involvement of sarcoidosis mimicking malignancy. Lymphoma and metastatic cancer proved a challenge to discard in differential diagnosis. He underwent multiple biopsies including tru-cut lung parenchyma and iliac bone biopsies. All of the biopsy specimens were reported as non-necrotising granulomatous inflammation. He underwent corticosteroid treatment. His response to the steroid treatment was insufficient so methotrexate was added to the corticosteroid therapy. Afterwards, clinical and radiological improvement was recorded. We aimed to highlight the utilization of taking new biopsies and histopathological re-evaluations in order to confirm the diagnosis and distinguish a benign disease from a malignant one.