Juvenile xanthogranuloma presenting as obstructive jaundice

Prasil, Pascale; Cayer, Sylvie; Lemay, Martin; Pelletier, Louise; Cloutier, Richard; Leclerc, Suzanne

doi:10.1016/s0022-3468(99)90567-5

Cited by 12 publications

(7 citation statements)

References 4 publications

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“…Systemic viral infection is another more commonly recognized cause of hemophagocytosis, but there was no evidence of viral infection on serology or viral cultures of the infant, nor in the placenta. It should be noted, however, that an association between JXG and cytomegalovirus infection has been reported [16].…”

Section: Discussionmentioning

confidence: 95%

Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure

Gilliam

Wiersma

et al. 2004

Pediatr Dev Pathol

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This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age. At autopsy, nodular tumor infiltrates of JXG were present throughout the liver, as well as in skin, abdominal lymph nodes, spleen, and pancreas.

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Section: Discussionmentioning

confidence: 95%

Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure

Gilliam

Wiersma

et al. 2004

Pediatr Dev Pathol

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“…Heintz et al [ 22 ] F/5mo Jaundice Head Whipple Resolution Liver 3. Prasil et al [ 23 ] NA/9mo Jaundice Head Mass excision Resolution – 4. Ueno et al [ 24 ] M/42y Belly pain Body (cyst) Distal pancreatectomy Resolution – 5.…”

Section: Discussionmentioning

confidence: 99%

Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature

Dai

Duan

et al. 2022

BMC Gastroenterol

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Background Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by infiltration of lipid-laden foamy macrophages within different tissues. Clinical manifestations of ECD are highly heterogeneous. Bone lesions are found in 80%-95% of patients, while extraosseous lesions usually involve the cardiovascular system, retroperitoneum, central nervous system (CNS), and skin. Pancreatic involvement in ECD has barely been reported. Case presentation A 29-year-old female initially presented with menoxenia, diabetes insipidus and diabetes mellitus. 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG-PET/CT) revealed hypermetabolic foci in the bilateral frontal lobe, saddle area, and pancreas. A 99mTc-MDP bone scrintigraphy scan revealed symmetrical increased uptake in distal femoral and proximal tibial metaphysis, which was confirmed to be osteosclerosis by high-resolution peripheral quantitative computed tomography. The patient underwent incomplete resection of the sellar mass. Histological examination of biopsies showed histiocytic aggregates, which were positive for S100 and negative for CD1a and CD207 on immunohistochemistry. Enhanced abdominal CT scan showed hypointense nodules within the body and tail of the pancreas. Endoscopic ultrasonography guided fine-needle aspiration (EUS-FNA) found no evidence of malignancy. She was diagnosed with ECD and treated with high-dose IFN-α. Repeated examinations at three-and eight-months post treatment revealed markedly reduction of both intracranial and pancreatic lesions. Conclusions ECD is a rare histiocytic neoplasm that can involve almost every organ, whereas pancreatic involvement has barely been reported to date. Here, we present the rare case of pancreatic lesions in ECD that responded well to interferon-α. We further reviewed reports of pancreatic involvement in histiocytic disorders and concluded the characteristics of such lesions to help diagnosis and treatment, in which these lesions mimicked pancreatic adenocarcinoma and caused unnecessary invasive surgeries.

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“…The presence of CMV positivity raised the question regarding the possible association with JXG. 18 Later, Heintz et al 17 described a case of a 5-month-old infant with obstructive jaundice found to have JXG of the pancreatic head who underwent Whipple procedure to establish a diagnosis. Dehner 1 in his published review article, reported a 2-month-old child with obstructive jaundice, diagnosed with pancreatic head JXG who also had cutaneous and subcutaneous involvement as well as a lung nodule.…”

Section: Discussionmentioning

confidence: 99%

“…21 For solitary lesions, like our case and the other reported cases of pancreatic JXG, surgical excision is thought to be curative. 1,17,18 Molecular and genetic testing is an essential part of the workup since there has been an association of juvenile myelomonocytic leukemia, neurofibromatosis type 1, and JXG. 1 The striking features of our case, in addition to the rarity of this entity, included the elevated tumor marker CA 19-9.…”

Section: Discussionmentioning

confidence: 99%

“…However, histopathology of the tumor did not show any CMV lesions. The presence of CMV positivity raised the question regarding the possible association with JXG 18. Later, Heintz et al17 described a case of a 5-month-old infant with obstructive jaundice found to have JXG of the pancreatic head who underwent Whipple procedure to establish a diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Juvenile Xanthogranuloma of the Pancreas in a Pediatric Patient Mimicking Pancreatic Neoplasm With High CA 19-9: Case Report and Literature Review

Al-Antary

Gupte

Poulik³

et al. 2021

Journal of Pediatric Hematology/Oncology

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Juvenile xanthogranuloma (JXG) is a rare, non-Langerhans cell histiocytosis. It is usually a benign and self-limiting condition. The most common sites are skin and soft tissue. Pancreatic involvement is extremely rare. We present an unusual case of a 13-month-old female child with JXG of the pancreas and elevated cancer antigen 19-9. JXG should always be considered as a differential diagnosis for pediatric patients presenting with a pancreatic mass, solid and/or cystic in nature. Therefore, avoiding unnecessary invasive diagnostic procedures.

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Juvenile xanthogranuloma presenting as obstructive jaundice

Cited by 12 publications

References 4 publications

Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure

Fatal Congenital Systemic Juvenile Xanthogranuloma with Liver Failure

Pancreatic involvement in Erdheim-Chester disease: a case report and review of the literature

Juvenile Xanthogranuloma of the Pancreas in a Pediatric Patient Mimicking Pancreatic Neoplasm With High CA 19-9: Case Report and Literature Review

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