Xanthogranulomatous Inflammation Research Articles

FP4.4 - Everything is not always as it seems – Discordance between cross-sectional imaging and pathology. A case report

Abstract Cross-sectional imaging of colonic pathology is usually correlated with other investigations such as optical colonoscopy (OC) prior to surgical treatment. In situations such as acute inflammation, this is not possible and surgery is undertaken based on cross-sectional imaging without histopathological confirmation. This case reports describes a 78-year-old female who presented with progressive shortness of breath associated with fatigue and unintentional weight loss. Initial bloods demonstrated a normocytic anaemia (Hb 105g/L, MCV 86fl), raised D-dimer (3460ng/mL) and raised C-reactive protein (133mg/L) with normal white cell count (8.6 x109/L). An unprepared computed tomography (CT) of the abdomen and pelvis revealed extensive bowel thickening in the caecum with radiological appearances consistent with a primary caecal malignancy. Preoperative colonoscopy was not performed as suspicion of perforation on CT imaging precluded the use of mechanical bowel preparation. Preoperative optimisation of the patient was commenced and an elective right hemicolectomy performed. The patient had an uneventful post-operative recovery. Results on histopathology inspection revealed chronic xanthogranulomatous inflammation but no macroscopic or microscopic evidence of dysplasia or malignancy. There continues to be an emerging role for the use of CT imaging, especially CT colonography, in the investigation and diagnosis of CRC. However, difficulty in distinguishing between malignant and non-malignant inflammation on CT endorses the quintessential place of OC with biopsy as the definitive investigation of CRC, especially if suspicion of CRC on unprepared CT. Xanthogranulomatous inflammation should be considered by clinicians, radiologists and pathologists as a differential diagnosis when CRC is suspected clinically and radiologically, without biopsy confirmation.

Xanthogranulomatous mastitis: a rare entity presenting with breast lump and mimicking fibroadenoma in a young female

Introduction and importance: Xanthogranulomatous mastitis is a rare chronic granulomatous inflammation of breast tissue, characterized by the presence of multinucleated giant cells, lipid-laden foamy macrophages, and cholesterol crystals. The breast is an extremely rare location for xanthogranulomatous inflammation. Case presentation: The authors describe a case of xanthogranulomatous mastitis in a 26-year-old female patient who presented with a 4×5 cm lump in her right breast. After ultrasonography (revealed a well-circumscribed hypoechoic lesion, with increased vascularity), and surgical consultation, an excisional biopsy was done, and sent for histopathologic examination, which showed features suggestive of xanthogranulomatous mastitis. Clinical discussion: Xanthogranulomatous mastitis, clinically as well as radiologically mimics some other conditions of breast like breast cancer, or fibroadenoma. Ultrasonography usually reveals a well-circumscribed hypoechoic lesion, with increased vascularity, and mammography usually shows BIRADs 3 to 5. Diagnosis is usually based on histopathologic findings of multinucleated giant cells, lipid-laden foamy macrophages, and cholesterol crystals. Treatment is usually surgical excision. Conclusion: Though xanthogranulomatous inflammations are rare in the breast, they must be considered a differential diagnosis in patients presenting with various breast symptoms like lump, pain, or features of breast malignancy.

Complex hysteroscopic diagnosis from a xanthogranulomatous endometritis to a uterine adenosarcoma after polyp resection: A case report

Uterine adenosarcoma is rare and can be associated with pelvic radiation and tamoxifen therapy in postmenopausal women. Symptoms are nonspecific but a recurrent polypoid formation is a characteristic clinical feature. Entities such as xanthogranulomatous inflammation

Xantho-granulomatous Orchitis: A Rare Cause of Scrotal Swellings

We aim to present an adult case of a rare cause of scrotal swelling i.e., xantho-granulomatous orchitis (XGO). The patient presented to us with a history of swelling and pain in the left scrotum for one month. There were no other associating symptoms and sexual and traumatic history were not present. Incision and drainage were planned for the patient under our care, however, because of ruptured tunica albuginea and dispersion of seminiferous tubules, left orchiectomy was performed and the specimen was sent for histopathological analysis. Xantho-granulomatous inflammation (XGI) is a relatively benign and infrequent condition and usually affects the 3rd-7th decade of life. The lipid-laden macrophages replace the destructed and necrotic normal tissue. The findings that are the hallmark of granulomatous diseases are necrosis and multinucleated giant cells which are not present in cases of XGI. It affects the testis, epididymis, spermatic cord, and bladder among genital organs.

Xanthogranulomatous appendicitis

Xanthogranulomatous inflammation is characterized histologically by a collection of lipid-laden macrophages admixed with lymphocytes, plasma cells, neutrophils, and often multinucleated giant cells with or without cholesterol clefts, that develops most often in the kidneys and gallbladder. But the involvement of vermiform appendix in the disease process is quite rare with only few cases are reported in literature. Xanthogranulomatous appendicitis is usually identified retrospectively on surgical pathology, that is associated with significant diagnostic and therapeutic dilemmas due to its variable presentation.

XANTHOGRANULOMATOUS CHOLECYSTITIS – A RARE HISTOLOGICAL VARIANT

Xanthogranulomatous cholecystitis is a rare presentation of chronic cholecystitis, characterized by xanthogranuloma, severe foam cells, and fibrosis. It can be an inducement of difficulty in cholecystectomy. In this case series, we describe four cases of cholecystitis which was diagnosed post-operatively to have xanthogranulomatous inflammation in the gallbladder on histopathological examination.

Xanthogranulomatous inflammation of the female genital tract: An 11-year single institutional study of 40 cases

Background and objectivesXanthogranulomatous inflammation (XGI) is a rare form of chronic inflammation that affects the female genital tract (FGT). The absence of a standard lexicon in the literature has contributed to the relative obscurity of this condition. We attempt to study this lesion with its various clinicopathological associations. MethodologyWe conducted an 11-year retrospective study of cases diagnosed with XGI of the FGT, analyzing relevant clinical and pathological parameters. ResultsOur study identified 40 cases reported as XGI. The mean age was 43.8 (+/− 11.8 SD) years. The most common clinical presentation was abdominal pain (27.5 %). Abdominal mass was seen in 37.5 % of which 22.5 % was primarily attributed to XGI and was not associated with malignancies. The most common site involved was adnexa(87.5 %), with rare involvement of myometrium(7.5 %) and endometrium(5 %). Adnexal involvement was either as tubo-ovarian masses or isolated ovary/fallopian tube involvement. XGI was also seen associated with other primary lesions of FGT like high-grade serous carcinoma(7.5 %) and mature cystic teratoma (7.5 %), while non-neoplastic associations included tubal gestation, foreign body, and Aspergillus infection in the ovary. Histologically, the infiltrate comprised of chronic inflammatory cells in all cases with additional acute inflammatory cells(60 %). Multinucleated giant cells were seen in 40 % of cases. Urine culture showed bacterial colonies in 23 % of cases. ConclusionsXGI of FGT is an extremely rare lesion and can present as isolated lesions or in association with other primary lesions of FGT. Adnexal involvement was more common than uterine XGI. It is essential to recognize this lesion because it can mimic malignancy and has a destructive nature.

Xanthogranulomatous appendicitis: A medical marvel

Xanthogranulomatous inflammation (XGI) is a rare but well- described type of chronic inflammation. It was in the genitourinary tract where it was first reported. Any organ can be involved but the most common sites are kidney followed by the gallbladder.Histologically, it can be characterized by a collection of lipids- laden macrophages along with inflammatory infiltrate, with or without cholesterol clefts.XGI is extremely uncommon in the appendix. Due to its atypical presentation, it is usually detected post- operatively. Sami Akbulut et al. published a comprehensive study in January 2021 and reported only 38 patients worldwide. Here, we present a case of a 20 – year old female who came to the OPD with typical signs of Acute Appendicitis diagnosed histopathologically as Xanthogranulomatous appendicitis

Urachal xanthogranuloma: a rare but important case presenting as a urachal mass

BackgroundA urachal mass is a relatively rare presentation to the urologists’ practice, often requiring radical surgical excision for a definitive diagnosis. Xanthogranulomatous inflammation of the urachus is an extremely rare entity with few cases reported worldwide, and to the best of our knowledge, no cases reported in the western world.Case presentationIn this case, a 55-year-old male patient presented with bothersome lower urinary tract symptoms and computed tomography findings demonstrating a urachal mass that was worrisome for urachal carcinoma. Following surgical intervention, histopathology revealed urachal xanthogranuloma. Post-operatively, the patient recovered well, and eventually, he had symptomatic and radiologic improvement.ConclusionThis case brings awareness to a rare presentation of a urachal mass—urachal xanthogranuloma. While operative intervention was both diagnostic and therapeutic, we highlight the challenge in differentiating between benign and malignant processes for urachal masses. Herein, we show the importance of including urachal xanthogranuloma in the differential diagnosis of a urachal mass to prevent further morbidity associated with the treatment of this disease.

Adult-Onset Asthma With Periocular Xanthogranuloma: Clinical-Histological Correlation.

A 48-year-old male presented with 10 years of gradually enlarging yellow eyelid lesions. Past medical history was notable for adult-onset asthma diagnosed 5 years ago. He denied pain, diplopia, bone/joint pain, or systemic findings such as weight loss or fatigue. His father had similar eyelid lesions. Preoperative photograph demonstrated bilateral upper eyelid yellowish lesions with prominent edema and tissue expansion inducing bilateral ptosis (Fig. 1A). MRI demonstrated eyelid inflammation, enlargement of the extraocular muscles, especially the right lateral rectus (Fig. 1B, white arrow), and enlargement and enhancement of lacrimal gland (Fig. 1C, black arrow). He desired debulking excisional biopsies with bilateral blepharoplasty. Intraoperative photos show significant soft tissue inflammatory infiltration, particularly the lacrimal gland (Fig. 1D, red arrows).FIG. 1.Histopathologically, xanthogranulomatous inflammation is characterized by diffuse inflammatory infiltrate composed of histiocytes and lymphocytes (Fig. 2A, H&E, 100×). Specifically, the presence of foamy macrophages (Fig. 2B, H&E, 400×, red arrows) and Touton Giant cells (Fig. 2C, H&E, 400×, black arrow) are the histological hallmarks of the disease. The differential diagnosis of xanthogranulomatous inflammation includes adult-onset xanthogranuloma, adult-onset asthma with periocular xanthogranuloma (AAPOX), Erdheim-Chester disease (ECD), necrobiotic xanthogranuloma (NBX), and juvenile xanthogranuloma.FIG. 2.The patient was diagnosed with AAPOX given the association with adult-onset asthma. While the vast majority of yellow eyelid lesions are clinically benign, ECD and NBX are must-not-miss diagnoses, given their potential life-threatening systemic manifestations. These include vital organ (heart/kidney/lung) involvement in ECD, and association with serum protein abnormalities and hematological malignancies in NBX.

Giant Cell Tumors With HMGA2::NCOR2 Fusion : Clinicopathologic, Molecular, and Epigenetic Study of a Distinct Entity.

Giant cell tumors (GCTs) with high mobility group AT-Hook 2 ( HMGA2 )::nuclear receptor corepressor 2 ( NCOR2 ) fusion are rare mesenchymal tumors of controversial nosology, which have been anecdotally reported to respond to CSFR1 inhibitors. Here, we performed a comprehensive study of 6 GCTs with HMGA2::NCOR2 fusion and explored their relationship with other giant cell-rich neoplasms. Tumors occurred in 4 females and 2 males ranging in age from 17 to 32 years old (median 24). Three lesions originated in subcutaneous soft tissue and 3 in bone. Tumor size ranged from 20 to 33mm (median 27mm). The lesions had a nodular/multinodular architecture and were composed of sheets of mononuclear "histiocytoid" cells with uniform nuclei intermingled with multinucleated giant cells. Mitotic activity was low and nuclear atypia and metaplastic bone were absent. Variable findings included necrosis, cystic degeneration, lymphocytic infiltrate (sometimes forming nodules), and xanthogranulomatous inflammation. On immunohistochemistry, all cases focally expressed pan-keratin and were negative with SATB2 and H3.3G34W. Whole RNA-sequencing was performed in all cases of GCT with HMGA2::NCOR2 fusion and a subset of giant cell-rich tumors (tenosynovial-GCT, n = 19 and "wild-type" GCT of soft tissue, n = 9). Hierarchical clustering of RNA-sequencing data showed that GCT with HMGA2::NCOR2 fusion formed a single cluster, independent of the other 2 entities. Methylome profiling showed similar results, but the distinction from "wild-type" GCT of soft tissue was less flagrant. Gene expression analysis showed similar levels of expression of the CSF1/CSFR1 axis between GCT with HMGA2::NCOR2 fusion and tenosynovial-GCT, supporting their potential sensitivity to CSFR1 inhibitors. Clinical follow-up was available for 5 patients (range: 10 to 64mo; median 32mo). Three patients (60%) experienced local recurrences, whereas none had distant metastases or died of disease. Overall, our study confirms and expands previous knowledge on GCT with HMGA2::NCOR2 fusion and supports its inclusion as an independent entity.

XANTHOGRANULOMATOUS APPENDICITIS-A RARE HISTOLOGICAL VARIANT

Xanthogranulomatous inflammation (XGI) is a chronic inflammatory process most commonly described in the case of gall bladder and kidney. The same occurring in the case of the vermiform appendix is a rare occurrence. Here, we describe two cases of appendicitis in which postoperatively histopathological examination revealed typical features of XGI in the appendix.

Correspondence on “Xanthogranulomatous inflammation caused by K. pneumonia and nocardiosis mimicking a uterine tumor and invading the ureter and colon: A case report and review of the literature”

Correspondence on “Xanthogranulomatous inflammation caused by K. pneumonia and nocardiosis mimicking a uterine tumor and invading the ureter and colon: A case report and review of the literature”

Xanthogranulomatous epididymo-orchitis: A single-institutional case series and systematic review.

Xanthogranulomatous inflammation is a rare nonneoplastic and chronic inflammatory process, characterized by proliferation of foamy macrophages resulting in damage and necrosis of the affected tissue. Involvement of the testis/epididymis by the disease is a rare event. A case series of four male patients diagnosed with xanthogranulomatous epididymitis/orchitis (XGEO) at our institute was reviewed. In addition, a systematic review of XGEO was carried out using PRISMA Guidelines 2020. Twenty-nine articles describing 38 patients of XGEO were included in the study. XGEO usually has a subacute or chronic presentation and affects male individuals in the 5th or 6th decades of life. The disease is also known to occur in the pediatric age group. The patients present with swelling, tenderness, or pain in the scrotal region. Bilateral involvement has also been documented. Thirty patients were known to have one or more causal risk factors including diabetes mellitus (23.7%), spinal cord injury/neuropathic bladder (7.9%), prostatectomy (7.9%), trauma (4.1%), and transurethral resection of prostate procedure (4.1%). Complications observed were scrotal fistula, adhesions, and abscess formation. Radiological features reported are nonspecific and include heterogeneous echotexture, hypoechoic areas, and/or scrotal wall collections. Bacterial microorganisms isolated from the affected tissue demonstrated the presence of Escherichia coli, Pseudomonas aeruginosa, and Staphylococcus aureus. Histological subtypes of XGEO are diffuse and focal. In the diffuse subtype, which is more common, there is extensive parenchymal destruction by inflammatory process accompanied by widespread ischemic necrosis. The mainstay of treatment in XGEO cases is surgical excision preferably orchidectomy. Conservative management has been attempted in young individuals and in patients with focal XGEO, but there is limited supporting evidence. We present data of four cases along with detailed systematic review of the disease examining its clinicopathological behavior and associated risk factors followed by operative approach.

Unusual Case of Xanthogranulomatous Mastitis with Florid Ductal Epitheliosis, Masquerading Clinically as Neoplastic Breast Lesion

Xanthogranulomatous inflammation is a rare form of chronic inflammation characterized by the presence of lipid-laden macrophages, multinucleate giant cells, and chronic inflammatory cells. It is an uncommon finding in the breast. Obstruction, infection with low-virulence organisms, defective lipid transport, autoimmune disorders, and allergic reactions have been implicated. Very few reports of xanthogranulomatous mastitis (XGM) have been reported in which coexistent benign and malignant lesions have been found. Clinical presentation of XGM is variable. Many patients present with pain and breast lumps. The lesions can mimic benign or malignant breast neoplasms clinically and radiologically which warrant a pathological diagnosis either by core or excisional biopsy. Histopathology examination is the key for diagnosis. Surgical excision is the choice of treatment. Larger lesions can be treated conservatively with antibiotics before surgical excision.

Xanthogranulomatous osteomyelitis of Tibia in a case of 43 years old male patient: A rare entity diagnosed in a tertiary care hospital

Xanthogranulomatous inflammation is a form of chronic inflammatory process with collection of foamy macrophages in sheets, often with presence of giant cell reaction. Here, we present a case of xanthogranulomatous osteomyelitis of tibia in a 43 years old patient, which is a rare pathological entity and possibly was reported first in 1984. The histopathological sections revealed dense collection of lympho-plasmacytic infiltrate in the marrow spaces with syncytial sheets of foamy macrophages admixed with numerous classical Touton giant cells.

Apendicitis xantogranulomatosa; un desafío diagnóstico: Caso Clínico

Introduction: Xanthogranulomatous inflammation is a chronic inflammatory process, rarely located in the appendix. A case of xanthogranulomatous appendicitis is presented. Clinical case. A 77-year-old male presents with intermittent pain in the right hemiabdomen and hyperthermia of one month's evolution. Physical examination showed a tumor in the iliac fossa and right abdomen. He underwent surgery for a probable incarcerated right inguinal hernia, acute perforated appendicitis was found, with inflammation and adhesions, purulent fluid in the right hemiabdomen and abdominal wall dissection. The histopathological result was xanthogranulomatous appendicitis. The evolution of the patient was favorable. Conclusion. Xanthogranulomatous appendicitis simulates a typical picture of acute appendicitis. Histopathologically, pathologies such as Crohn's disease, malacoplakia and tuberculous appendicitis were ruled out.

Interval Appendectomy Specimens.

Patients with perforated appendicitis are often managed with antibiotic therapy followed by a delayed appendectomy. Histologic features of such specimens have been incompletely described, especially in the recent literature. To describe the histomorphology of interval appendicitis with a focus on features that could mimic important conditions, such as infections, Crohn disease, and mucinous neoplasms. Histologic evaluation of 100 interval appendectomy specimens with clinical and radiologic correlation. A total of 54 of the 100 patients (54%) had radiologic evidence of appendiceal perforation, and 97% were treated with intravenous and/or oral antibiotic therapy prior to appendectomy. Percutaneous drains were placed in 34 cases (34%). Common histologic findings included mural eosinophilic infiltration (54%), periappendiceal fibrosis (54%), and xanthogranulomatous inflammation (31%). Periappendiceal fibrosis was frequent among patients with radiologic evidence of perforation. Nine cases (9%) featured pulse granulomata associated with fecal material. Epithelioid granulomata were detected in 6% of cases and were confined to mucosal lymphoid follicles in all cases. Only 4 of these were accompanied by mural lymphoid aggregates that raised the possibility of Crohn disease. Changes mimicking mucinous neoplasms were more common: 14% of cases (14 of 100) displayed goblet cell hyperplasia, 15% (15 of 100) contained diverticula, and 16% (16 of 100) showed mural or periappendiceal mucin pools. Although interval appendectomy specimens occasionally contain inflammatory infiltrates that mimic infections and/or Crohn disease, changes that can be confused with mucinous neoplasms are more frequently encountered.

Interval appendicitis shows histological differences from acute appendicitis and may mimic Crohn disease and other forms of granulomatous appendicitis.

While patients presenting with clinical signs and symptoms of acute appendicitis (AA) often receive surgical intervention shortly after presentation, certain patients may instead receive non-operative management initially, with appendectomy later. The histology of such interval appendicitis (IA) has only been described in small series. Also, we have noticed a recent increase in the incidence of IA specimens at our institution. We identified appendectomy specimens in our department during 2018 with available haematoxylin and eosin slides and electronic clinical data, and evaluated multiple histological findings. Cases were then divided into AA and IA, based on clinical history (AA if the patient presented to the hospital within 1 week of symptom onset and underwent appendectomy within 48 h; IA if appendectomy was delayed at least 1 week). Changes between groups were compared. The cohort included 165 cases (125 AA, 40 IA). Findings significantly more common in AA included mucosal acute inflammation, mural acute inflammation and acute serositis. Findings significantly more common in IA included Crohn-like mural inflammation, mural fibrosis, goblet cell hyperplasia, granulomas, xanthogranulomatous inflammation, haemosiderin-laden macrophages and granulation tissue. The rate of IA in 2018 (24%) was noticeably higher than in previous years. Acute inflammatory changes are more common in AA but can remain present in IA. Mural fibrosis, serosal adhesions, haemosiderin-laden macrophages and granulation tissue suggest IA. Granulomas and xanthogranulomatous inflammation can also be seen in IA, and Crohn-like mural inflammation is not uncommon. These histological patterns can guide signout and prevent diagnostic errors, particularly when clinical information is unavailable.

COVER (PMS 180&K) (p/u from previous issue w/updates)

Xanthogranulomatous orchitis is an extremely rare inflammatory nonneoplastic lesion of the testis. We report a case of a 13-year-old adolescent boy who presented a painless left hemiscrotal swelling. The subsequent ultrasonography and magnetic resonance imaging revealed the presence of abnormal expanding tissue located in both testes and spermatic cord, reaching the internal inguinal ring. Testicular tumor markers were normal. The frozen section examination of the surgical specimen showed only inflammatory tissue and not neoplastic tissue. No orchiectomy was performed. Definitive histopathologic diagnosis was xanthogranulomatous inflammation. To our knowledge, this is the youngest case of xanthogranulomatous orchiepididymitis and funiculitis found in medical literature.