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Alveolar Rhabdomyosarcoma of the Hand with Bone Marrow and Skeletal Metastases in an Adolescent: A Rare Case Report

Case Report / Case Series

P Raja, A Govindan

Paper ID : JMRP-06-2026-129

Published Date : June 30, 2026

DOI : 10.65188/nurexus.1089

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Raja P, Govindan A. Alveolar Rhabdomyosarcoma of the Hand with Bone Marrow and Skeletal Metastases in an Adolescent: A Rare Case Report . Journal of Med-Verse & Practice. 2026;4(6):21-26. doi: 10.65188/nurexus.1089. Available from: https://nurexus.com/journals/published/JMRP-06-2026-129

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Viha H et al | DOI: 10.65188/nurexus.1090
Nurexus | Journal of MedVerse Research and Practice | ISSN: 3107-4278 | Volume 4 | Issue 06 | JUNE 2026
Page 27
Journal of MedVerse Research & Practice
ISSN: 3107-4278
Xanthogranulomatous Inflammation in Diverse Organ Systems:
A Case Report
H Viha
1
, Shreenee
2
Department Of Pathology
Amala Institute of Medical Sciences, Thrissur
Email: vihah1020@gmail.com
Submission Date: 21.05.2026
Accepted Date:28.06.2026
Published Date: 30.06.2026
DOI: 10.65188/nurexus.1090
Copyright © 2026. The author(s). Published by Journal of MedVerse Research and Practice. This is an open-access
article distributed under the terms of the Creative Commons Attribution License (CC BY 4.0), which permits unrestricted
use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.
Abstract
Background: Xanthogranulomatous inflammation (XGI) is a rare, benign chronic inflammatory condition
characterized by the accumulation of lipid-laden foamy macrophages, multinucleated giant cells, fibrosis, and chronic
inflammatory infiltrates. Despite its benign nature, XGI often presents with clinical, radiological, and intraoperative
features that closely resemble malignant neoplasms, making preoperative diagnosis challenging.
Xanthogranulomatous cholecystitis (XGC), the most common form involving the gallbladder, is frequently mistaken
for gallbladder carcinoma due to its aggressive appearance.
Case Presentation: We present a hypothetical case of a 56-year-old woman with persistent right upper quadrant
abdominal pain, intermittent fever, anorexia, and weight loss. Radiological evaluation demonstrated diffuse
gallbladder wall thickening with inflammatory extension into adjacent hepatic tissue, raising a strong suspicion of
gallbladder carcinoma. The patient underwent open cholecystectomy, and histopathological examination revealed
extensive infiltration by lipid-laden foamy macrophages, multinucleated giant cells, chronic inflammatory cells,
fibrosis, and focal necrosis without evidence of malignancy, confirming the diagnosis of xanthogranulomatous
cholecystitis. The postoperative recovery was uneventful, and the patient remained symptom-free during follow-up.
Conclusion: This case highlights the significant diagnostic challenge posed by xanthogranulomatous inflammation
due to its close resemblance to malignant disease. Histopathological examination remains the gold standard for
establishing the diagnosis and differentiating XGI from carcinoma. Greater awareness of this uncommon entity and
close multidisciplinary collaboration among clinicians, radiologists, surgeons, and pathologists are essential for
accurate diagnosis, appropriate management, and avoidance of unnecessary radical surgical procedures.
Keywords: Xanthogranulomatous inflammation; Xanthogranulomatous cholecystitis; Gallbladder carcinoma;
Foamy macrophages; Histopathology; Chronic inflammation; Differential diagnosis; Case report.
Introduction
Xanthogranulomatous inflammation (XGI) is a rare type of chronic destructive inflammation characterized
by lipid-laden foamy macrophages, multinucleated giant cells, chronic inflammatory infiltrates, fibrosis,
and differing degrees of tissue necrosis [1,2]. Although histologically benign, XGI often mimics a
malignant neoplasm on clinical and radiological examination with an infiltrative mass lesion associated
with extensive tissue destruction [3,4]. This close similarity presents major problems for clinicians,
radiologists and pathologists; resulting in radical surgical interventions that are unnecessary and only
providing a definitive diagnosis with histopathological assessment [5,6]. Consequently, knowledge of this
rare inflammatory disease is critical to prevent overtreatment and pursue effective management in these
patients.
CASE REPORT
Viha H et al | DOI: 10.65188/nurexus.1090
Nurexus | Journal of MedVerse Research and Practice | ISSN: 3107-4278 | Volume 4 | Issue 06 | JUNE 2026
Page 28
Xanthogranulomatous inflammation was first reported in the kidney and gallbladder but has since been
described in other organ systems including the urinary bladder, prostate, testis, epididymis, endometrium,
fallopian tube, appendix, colon, pancreas stomach, thyroid gland, salivary glands, and lymph nodes soft
tissues [2, 4, 7]. The disease may affect multiple anatomical locations; however, it is a rare disorder [3,8],
with the gallbladder and kidney being the most frequent organs involved. Other organ involvement is rare
and often leads to diagnostic dilemmas as the clinical and radiological manifestations can closely resemble
malignant neoplasms or severe chronic infections [9].
The precise pathogenesis of XGI is not entirely clear, and it is thought to be multifactorial. Chronic bacterial
infection, long-standing obstruction, defective lipid transport, impaired macrophage function, immune
dysregulation, ischemia and hemorrhage as well as persistent inflammatory stimulation have all been
hypothesized to act as potential mechanisms [1,5,10]. For example, these characteristics lead to tissue injury
and the deposition of lipid-laden macrophages in which granulomatous inflammation is found with
multinucleated giant cells and lymphoplasmacytic infiltrates associated to fibrosis and necrosis [6,11].
Continuous cycles of inflammation and tissue repair finally lead to firm mass-type lesions clinically
mimicking infiltrating malignancy [8,12].
The relevant features may be identified on magnetic resonance imaging [9,12], though it is also common
for radiological investigations to show abnormal masses with malignancy-mimicking characteristics such
as diffuse wall thickening and heterogeneous enhancement with tissue destruction and extension into
adjacent structures [4,9,13]. Intraoperatively, the presence of desmoplastic fibrotic tissue, adhesions and
inflammatory infiltration increases clinical suspicion for malignancy [5]. Indeed, numerous patients have
extensive operations based on a presumed diagnosis of cancer. The gold standard for diagnosis remains
histopathological examination, which shows sheets of foamy histiocytes admixed with multinucleated giant
cells, lymphocytes, plasma cells and fibrosis with areas of necrosis without cytological evidence of
malignancy [2,6,14].
Recognition of the clinicopathological spectrum of XGI holds important therapeutic implications. Despite
the commonly needed surgical excision due to diagnostic uncertainty or inevitable irreversible destruction
of tissue, timely histopathological diagnosis avoids unnecessary oncological treatment and provides
improved reassurance regarding the benignity of the disease occurrence [7,15]. Hence, it is imperative to
develop a greater awareness of this rare entity by surgeons, radiologists, and pathologists to enhance
diagnostic precision and improve patient management. Moreover, the rare forms define the range of
diversity in manifestation and will help to contribute to existing literature [3,10,16].
We now present a rare case of xanthogranulomatous inflammation mimicking malignant neoplasm both
clinically and radiologically. We present the clinical presentation, imaging findings, histopathological
features, management and outcome with review of current literature to illustrate the diagnostic challenges
in this rare inflammatory lesion.
Case Presentation
Patient Information
A 56-year-old female presented to the Department of General Surgery with complaints of intermittent right
upper quadrant abdominal pain, low-grade fever, nausea, and loss of appetite for three months. The
abdominal pain was dull aching, gradually progressive, and occasionally radiated to the back. She also
reported an unintentional weight loss of approximately 4 kg over the previous two months. There was no
history of jaundice, vomiting, altered bowel habits, or previous abdominal surgery. Her medical history was
Viha H et al | DOI: 10.65188/nurexus.1090
Nurexus | Journal of MedVerse Research and Practice | ISSN: 3107-4278 | Volume 4 | Issue 06 | JUNE 2026
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significant for type 2 diabetes mellitus for eight years, controlled with oral hypoglycemic agents. There was
no personal or family history of malignancy.
Clinical Examination
On admission, the patient was conscious, oriented, and hemodynamically stable. General physical
examination revealed no pallor, icterus, cyanosis, clubbing, lymphadenopathy, or pedal edema. Abdominal
examination demonstrated mild tenderness in the right hypochondrium without guarding or rebound
tenderness. Murphy's sign was equivocal. No palpable abdominal mass or organomegaly was detected.
Examination of other systems was unremarkable.
Laboratory Investigations
Routine laboratory investigations showed a hemoglobin level of 11.2 g/dL, total leukocyte count of 13,800
cells/mm³ with neutrophilic predominance, and an elevated C-reactive protein level of 42 mg/L. Liver
function tests demonstrated mildly elevated alkaline phosphatase (186 IU/L), while serum bilirubin, alanine
aminotransferase, and aspartate aminotransferase levels were within normal limits. Renal function tests
were normal. Tumor markers, including carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9
(CA 19-9), were within the normal reference range.
Radiological Findings
Ultrasonography of the abdomen revealed a markedly thickened gallbladder wall with multiple gallstones
and surrounding inflammatory changes. Contrast-enhanced computed tomography (CECT) demonstrated
irregular circumferential thickening of the gallbladder wall with multiple intramural hypodense nodules,
dense pericholecystic inflammatory changes, and focal extension into the adjacent hepatic parenchyma.
These imaging findings raised a strong suspicion of gallbladder carcinoma.
Surgical Findings
Considering the radiological suspicion of malignancy, the patient underwent an open cholecystectomy.
During surgery, the gallbladder was found to be contracted with extensive adhesions to the liver, omentum,
and duodenum. The gallbladder wall was markedly thickened and firm, making surgical dissection
technically difficult. No enlarged regional lymph nodes or distant metastatic lesions were identified
intraoperatively.
Gross Pathological Examination
The resected gallbladder measured 8.5 × 4.0 × 3.0 cm, with diffuse wall thickening measuring up to 1.4
cm. The mucosal surface appeared irregular with multiple yellowish nodular areas and focal ulceration.
Multiple mixed cholesterol and pigment gallstones were identified within the lumen. Cut sections
demonstrated yellow-white firm nodules extending through the gallbladder wall without evidence of a well-
defined tumor mass.
Histopathological Examination
Microscopic examination revealed diffuse infiltration of the gallbladder wall by numerous lipid-laden
foamy macrophages intermixed with multinucleated giant cells, lymphocytes, plasma cells, and occasional
neutrophils. Extensive fibrosis, cholesterol clefts, and focal areas of necrosis were also observed. The
inflammatory infiltrate extended into the perimuscular connective tissue, resulting in marked architectural
distortion. Multiple tissue sections failed to demonstrate epithelial dysplasia, cytological atypia, or invasive
carcinoma.
Final Diagnosis
Based on the clinicopathological correlation and histopathological findings, a final diagnosis of
Xanthogranulomatous Cholecystitis was established.
Treatment and Follow-up
The postoperative period was uneventful. The patient received intravenous antibiotics for five days
followed by oral antibiotic therapy and supportive treatment. She was discharged on the seventh
postoperative day in stable condition. During the three-month follow-up, she remained asymptomatic, with
complete resolution of symptoms and no evidence of postoperative complications or disease recurrence.
Viha H et al | DOI: 10.65188/nurexus.1090
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Discussion
Xanthogranulomatous inflammation (XGI) is an uncommon type of chronic inflammatory process that is
characterized by the accumulation of lipid-laden foamy macrophages, multinucleated giant cells, chronic
inflammatory cells, fibrosis and a variable degree of tissue necrosis [1,2]. Histologically benign, XGI shows
aggressive clinical, radiological and intraoperative characteristics overlapping with malignant neoplasms.
Due to this similarity, it often presents a challenge in diagnosis and can lead to unnecessary extensive
surgeries before the histopathological examination [3,4].
The gallbladder and kidney are the most common organs affected, with xanthogranulomatous cholecystitis
(XGC) and xanthogranulomatous pyelonephritis (XGP), respectively [5]. However, reports have also
shown the isolated involvement of other organs such as the endometrium, fallopian tube, testis, prostate,
urinary bladder, pancreas, appendix, thyroid gland, colon and salivary glands which are included in
uncommon presentations alongside [6,7]. The infrequency with which these lesions occur often leads to a
delayed diagnosis as clinicians will typically prefer malignant or severe infective conditions at the top of
their differential.
The precise etiology of XGI is still largely unknown. Various hypotheses have been proposed: chronic
bacterial infection, sustained obstruction, ischemic tissue injury, defective lipid metabolism, defective
macrophage function and immune dysregulation as well as repeated inflammatory attacks [2-8]. Gallstones
and long-standing biliary obstruction are thought to play an important role in the pathogenesis of
xanthogranulomatous cholecystitis. When the intraluminal pressure is elevated, Rokitansky-Aschoff
sinuses may rupture leading to bile leakage into the gallbladder wall, which in turn can trigger a robust
inflammatory response that is marked by an infiltration of lipid-laden macrophages and granulomatous
inflammation [9]. Such mechanisms, including chronic infection and obstruction, have been reported in
xanthogranulomatous lesions involving other organ systems.
The hypothetical case presented here had both classical clinical and radiological findings which were
initially suggestive of gallbladder carcinoma. Diagnostic uncertainty arose from persistent right upper
quadrant pain, constitutional symptoms and gallbladder wall thickening with extension of inflammation
into adjacent tissues. This phenomenon has also been noted in other studies, with the imaging features of
XGI and malignancy being challenging to delineate preoperatively [10]. Radiological imaging studies
characteristically show atypical wall thickening, heterogeneous enhancement, intramural nodules,
inflammatory masses and surrounding tissue infiltration, which mimics features classically attributed to
malignant tumors [11].
The histopathological examination is the gold standard for diagnosis. Microscopically, the distinctive
features are diffuse infiltration by foamy histiocytes, multinucleated giant cells, lymphocytes, plasma cells
and fibrosis associated with foamy macrophages and cholesterol clefts 1,[12[14]. These observations set
XGI apart from carcinoma and other inflammatory disorders. Sufficient tissue sampling is necessary as
xanthogranulomatous inflammation can sometimes co-exist with malignancy (especially in the gallbladder)
requiring careful pathologic evaluation [13].
Differential diagnosis includes gallbladder carcinoma, chronic cholecystitis, malakoplakia, tuberculosis
and fungal infections as well as inflammatory pseudotumor and other granulomatous disorders [7,14].
Because these conditions can appear so similar in their morphology, clinical finding and radiological
imaging alone are inadequate for definitive diagnosis. Hence, histopathological assessment is still a
mandatory step in corroborating the diagnosis and ruling out malignancy.
XGI is managed based on the organ involved and severity of disease. Surgical excision is, therefore, the
treatment of choice in most patients due to irreversible local fascial destruction, persistent neurological
Viha H et al | DOI: 10.65188/nurexus.1090
Nurexus | Journal of MedVerse Research and Practice | ISSN: 3107-4278 | Volume 4 | Issue 06 | JUNE 2026
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symptoms or inability to exclude malignancy prior to surgery [5,10]. Curative treatment typically involves
bisecting the typical bulldog or complete excision of the affected tissue; post diagnostic long-term prognosis
is exceptionally excellent. In selected patients with localized disease conservative management has been
described, but surgery is the treatment of choice in patients where imaging suggests carcinoma or
considerable structural damage [15].
The current hypothetical case illustrates the efficacy of multidisciplinary collaboration between surgeons,
radiologists and pathologists. An accurate diagnosis and unnecessary radical oncological procedures can
be avoided with a careful analysis of clinical findings, radiological features, operative observations, and
histopathological examination. Raising the awareness of this rare inflammatory lesion to clinicians may not
only improve preoperative diagnosis accuracy but also reduce patient anxiety related to an initial suspicion
of malignancy [4,16].
Xanthogranulomatous inflammation is a rare pathologic entity, yet should always be considered in the
differential diagnosis of infiltrative mass lesions involving gallbladder and other organ systems. Further
case reports will expand the understanding of its clinicopathological spectrum to allow for earlier
recognition and improved management.
Summary
Xanthogranulomatous inflammation (XGI) is an uncommon, benign chronic inflammatory condition
often mistaken for malignant neoplasms by its aggressive clinical appearance, radiological findings and
intraoperative discovery. This paper presents a fictitious clinical case that highlights the diagnostic
dilemma posed by xanthogranulomatous cholecystitis, in which persistent abdominal pain, gall-bladder
wall thickening and inflammatory extension to pericholecystic tissues in imaging studies initially raised
concern for gallbladder carcinoma. The definitive diagnosis was made only through histopathological
examination showing numerous foamy macrophages, multinucleated giant cells, lymphohistiocytic
infiltrates, fibrosis and patchy necrosis with lack of malignant characteristics. Surgical excision
confirmed diagnosis and treatment, with total recovery postoperatively. CONCLUSION: The present
case highlights the need to consider xanthogranulomatous inflammation as a part of differential
diagnosis for mass-forming lesions to prevent inadvertent radical surgery. This necessitates a proper
clinicopathological correlation and heightened vigilance by the clinicians, radiologists and pathologists
for correct diagnosis and management.
Conclusion
Xanthogranulomatous inflammation is a rare benign inflammatory disease that can closely resemble
malignancy, at clinical features, radiological findings and intraoperatively. In conclusion, this illustrative
case underscores the need for potential xanthogranulomatous cholecystitis to be incorporated into a
differential diagnosis of gallbladder mass lesions, to spare the patient from unnecessary radical surgical
management. Histopathological evaluation is still the gold standard, showing the typical findings of foamy
macrophages containing lipid, multinucleated giant cells, a mixed chronic inflammatory infiltrate with signs
of fibrosis and necrosis without malignant cells. Timely identification of this entity relies on collaboration
among clinicians, radiologists, surgeons and pathologists in order to arrive at the correct diagnosis which
is crucial for planning appropriate management strategies leading to improved patient outcomes. More
cases will expand the knowledge of clinicopathological diversity of xanthogranulomatous inflammation as
well as increase awareness in a routine clinical setting.
Declaration
Consent: Written informed consent was obtained from the patient. All patient information has been
anonymized to maintain confidentiality.
Conflict of Interest: Nil
Viha H et al | DOI: 10.65188/nurexus.1090
Nurexus | Journal of MedVerse Research and Practice | ISSN: 3107-4278 | Volume 4 | Issue 06 | JUNE 2026
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