International Journal of Health Sciences and Research Vol.10; Issue: 7; July 2020 Website: www.ijhsr.org Case Report ISSN: 2249-9571

Xanthogranuloma of Abdominal Wall - A Rare Case Report

Ruchi Khadayate1, Reeta Dhar2, Siddhi Karale1

1Second year Post- Graduate student, 2 Professor, Department of Pathology, MGM Medical College & Hospital, Kamothe, Navi Mumbai

Corresponding Author: Reeta Dhar

ABSTRACT

Xanthogranulomatous inflammation is a rare inflammatory process characterized by proliferation of and touton giant cells. It can simulate a neoplastic process. The causative agent can be any infectious process or any causative organism, inflammatory conditions or lysosomal storage disorders like Niemann-Pick, Gaucher’s disease etc. Xanthogranulomatous inflammation comes in the category of Non- histiocytosis. Pathologists should know the histologic features of this histiocytic disorder to categorize into and also help in patient management.

Keywords: Xanthogranuloma, Abdominal wall, Non-Langerhans cell histiocytosis

INTRODUCTION- perisplenic and interbowel pelvis with Xanthogranuloma is the most multiple septation was noted suggestive of common type of Non-Langerhans cell Chronic Ascites. In view of radiological histiocytosis. It is caused by proliferation of findings, patient was posted for Exploratory histiocytes (). Two of the most Laparotomy Surgery in view of important Histiocytic disorders are Juvenile Pyoperitoneum and Appendicitis. During Xanthogranuloma and Langerhans cell Exploratory Laparotomy, Appendix with histiocytosis. Juvenile Xanthogranuloma is part of peritoneum and tissue connecting the a disease of infancy or childhood and most anterior abdominal wall (umbilicus ) to the commonly presents as cutaneous nodules bladder(muscle tract)- was resected and sent over head and neck region. to Histopathology section of the Central Xanthogranulomas occur in adult life also Laboratory of MGM Medical College, with involvement of deep soft tissue, testis, Kamothe, Navi Mumbai. pericardium, lung, kidney, retroperitoneum Gross Finding- and spleen. (1) Received three specimens as follows- 1. Labelled as Appendix- Single, tubular, CASE REPORT grey white to grey brown, soft to firm A 71 yrs old male patient came to tissue piece measuring 6cm in length. casualty with abdominal pain, distension External surface- congested. and difficulty in passing urine and stools On cutting the tissue- Lumen identified. since 4 days. Patient is chronic alcoholic 2. Labelled as muscle tract- Multiple, grey and smoker since past 15 years. Patient was brown, soft to firm tissue pieces largest admitted under surgery and full workup of measuring 3.5x2.5x1cm and smallest the patient was done. The complete measuring 1cm in length. count revealed Hb- 9.8gm/dl, TLC- 3. Labelled as Omentum- Single, grey- 35000cells/cumm, Plt-1.90 lakhs/cumm. On yellow, soft to firm tissue piece USG abdomen, free fluid in perihepatic, measuring 3.5x1.0x0.5cm.

International Journal of Health Sciences and Research (www.ijhsr.org) 91 Vol.10; Issue: 7; July 2020 Ruchi Khadayate et.al. Xanthogranuloma of abdominal wall- a rare case report

Microscopic Finding- noted. The infiltrate is seen extending to Appendix and Omentum revealed surrounding muscle and fat tissue. Focal features of Acute Appendicitis (Transmural collections of neutrophilic infiltrate with acute inflammatory infiltrate). No and lymphocytes with congested multinucleate giant cells or granulomas blood vessels and hemorrhage are seen. No noted in the sections studied. well formed epithelioid granuloma or Histological features from the caseous necrosis were noted. No lining muscle tract revealed ill circumscribed, non- epithelium or cystic structure identified in capsulated lesion comprising of the sections studied. predominantly fibrocollagenous tissue infiltrated by mixed inflammatory infiltrate Special stains with Ziehl-Neelsen, Fite- composed of lymphocytes, plasma cells, Faraco, Periodic Acid–Schiff, and Grocott histiocytes and numerous multinucleate Methenamine Silver Nitrate stains were Touton type of giant cells along with negative hence no evidence of any Koch’s bundles of skeletal muscles and parasite or fungal infections were myofibroblasts. Few focal collections of demonstrated. cells and histiocytes with foamy Diagnosis of Inflammatory lesion to slightly eosinophilic cytoplasm are also suggestive of Xanthogranuloma was given.

Figure-1 Figure-2 Figure 1 & 2- Micrographs show numerous multinucleate giant cells (Red arrows) along with collagenous tissue, skeletal muscle bundles (Green arrow) and inflammatory infiltrate. (H & E stain 4X)

10 X 10X Figure-3 Figure-4 Figure-3&4- Micrographs shows numerous Touton type of multinucleate giant cells (Arrow head) along with numerous xanthoma cells (foamy macrophages (Arrows) and inflammatory infiltrate comprising of lymphocytes, plasma cells, eosinophils and . (H&E stain 10X)

International Journal of Health Sciences and Research (www.ijhsr.org) 92 Vol.10; Issue: 7; July 2020 Ruchi Khadayate et.al. Xanthogranuloma of abdominal wall- a rare case report

10 10X X Figure-5 Figure-6 Figure 5 – Micrographs shows eosinophils (arrow) in the inflammatory infiltrate with fibroblastic proliferation. (H&E stain 10X) Figure 6- Micrographs shows areas of haemorrhages (arrow head) along with dilated congested blood vessel and foamy macrophages . (H&E stain 10X) formation, or hemorrhage. (4) There can be capillary proliferation, collagen formation, and hemorrhages. The histiocytes in close proximity to the hemorrhage engulf the erythrocytes (erythrophagocytosis), forming lipid-laden histiocytes (xanthoma cell). This inflammatory process results in granulation and necrotic debris. (5) This process eventually leads to replacement of the parenchymatous cells with abundant xanthoma cells, fibrin, collagen deposition and tissue destruction. Xanthogranulomatous pyelonephritis, cholecystitis and of visceral Figure -7 organ and genitourinary organs are a very Figure-7- Micrographs shows Touton showing multiple nuclei forming a ring surrounded by foamy cytoplasm making the known entities. The causative mechanism is cytoplasm visible around the nuclei. And also seen are foamy the extensive inflammatory response against macrophages and lymphocytes. (H&E stain 40X) the various mechanical and microbiological

insults to the particular organ. They are DISCUSSION known to involve the surrounding structures Histiocytes are considered to arise like the mesenteries simulating aggressive from stem cell precursors in the bone process leading to abscess formation marrow that undergo differentiation toward mimicking a tumour mass radiologically. (6) the antigen-presenting cell (class I) or (2) Various organs of the body are - (class II) lineage. known to be involved with kidney and the Xanthogranulomatous inflammation is a gall bladder being the commonest ones. (7) rare aggressive inflammatory conditions that Other predominantly involved sites, which can be caused by infection, inflammation, have been reported are endometrium, histiocytic processes, or inherited disorders [3,4] epididymis, fallopian tubes, bone, skin, and are not pathognomonically specific appendix, urinary bladder, thyroid and but are characterized by admixture of adrenal glands. (8,9) Spillage of gall stone or granular, eosinophilic, periodic acid- Schiff–positive histiocytes, foamy perforation of appendix or involvement of macrophages, and plasma cells other visceral organs causes spread to the accompanied by a varying degree of surrounding structures is a known cause of suppurative inflammation, abscess xanthogranulomatous inflammation of the abdominal cavities. Exact pathogenesis of

International Journal of Health Sciences and Research (www.ijhsr.org) 93 Vol.10; Issue: 7; July 2020 Ruchi Khadayate et.al. Xanthogranuloma of abdominal wall- a rare case report xanthogranulomatous inflammation is not REFERENCES yet known but different hypothesis are given 1. James W. Patterson. Chapter-2 -Non melanocytic cutaneous tumour, Steinberg’s like defective lipid transport, disorders of th neutrophilic chemotaxis, lymphatic diagnostic surgical pathology :6 edition obstruction and specific immune response vol-1 page-67 2. Cline MJ. Histiocytes and histiocytosis. towards Proteus and E. coli. (10) Blood. 1994;84:2840–2853 Systemic Xanthogranulomatous 3. Zaveri J, Quan L, Yarmish G, Neuman J. process is a rare disorder of non Langerhans More than just Langerhans cell cell histiocytosis. They are comprised of histiocytosis: a radiologic review of Erdheim-Chester disease (ECD), Rosai- histiocytic disorders. RadioGraphics 2014; Dorfman disease (RDD), hemophagocytic 34:2008–2024 lymphohistiocytosis (HLH), and juvenile 4. Cozzutto C, Carbone A. The xanthogranuloma (JXG). These disease xanthogranulomatous process: show multisystem involvement with xanthogranulomatous inflammation. Pathol diagnosis is made on radiological, Res Pract 1988; 183:395–402 histopathological and immunohistochemical 5. Hayes WS, Hartman DS, Sesterbenn IA. From the archives of the AFIP: findings. (11) xanthogranulomatous pyelonephritis. In our case, peritonitis was present RadioGraphics 1991; 11:485–498 but the appendix, gall bladder and omentum 6. Kelsey S et al Spectrum of did not show xanthogranulomatous Xanthogranulomatous Processes in the inflammation and also there was no Abdomen and Pelvis: A Pictorial Review of evidence of perforation of the abdominal Infectious, In ammatory, and Proliferative viscerae. Serous and pus collection was Responses.AJR 2017; 208:475–484 noted over the bladder with a tract like 7. Franco V, Aragona F, Genova G, et al. tissue from the bladder to anterior Xanthogranulomatous cholecystitis. abdominal wall. No signs of obstruction Histopathological study and classification. were noted in bowel. All though Patient Pathol Res Pract. 1990;186:383–90. 8. Oh YH, Seong SS, Jang KS, et al. gave history of gastrointestinal ulcers for Xanthogranulomatous in ammation which he was operated 40 yrs back. But no presenting as a submucosal mass of the current symptoms or relevant history was sigmoid colon. Pathol Int. 2005;55:440– present. 44. 9. Gray Y, Libbey NP. Xanthogranulomatous CONCLUSION salpingitis and oophoritis: a case report and We would like to conclude by saying review of the literature. Arch Pathol Lab that our case presented with intramuscular Med. 2001;125:260–63. xanthogranulomatous inflammation which 10. Gaurav Kochhar et al., was mostly an acquired disorder with no Xanthogranulomatous Appendicitis with a evidence of xanthogranulomatous fulminant course: Report of a case. Journal inflammation in the gall bladder, appendix of Clinical and Diagnostic Research. 2014 or any other resected tissue. Intraoperative Dec, Vol-8(12): ND01-ND02 11. John D. Reith. Bones and joints. Rosai and finding of the muscle tract connecting the Ackerman’s Surgical Pathology, First South anterior abdominal wall to bladder Asia edition. Ch-40, Pg no-1788-1789. suspecting it to be urachus but no lining epithelium was seen in the multiple sections How to cite this article: Khadayate R, Dhar R, from the resected tissue. Karale S. Xanthogranuloma of abdominal wall- a rare case report. Int J Health Sci Res. 2020; 10(7):91-94.

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International Journal of Health Sciences and Research (www.ijhsr.org) 94 Vol.10; Issue: 7; July 2020