Synchronous Colon and Pancreatic Rosai-Dorfman Disease

Noggle, Emily; Ortanca, Ibrahim; Clark, Ian A.; Yadak, Nour; Glazer, Evan S.

doi:10.1177/0003134820950294

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…Liu et al [ 45 ] F/71y Fullness Tail Distal pancreatectomy Resolution – 11. Emily et al [ 46 ] F/40y Belly pain Tail Distal pancreatectomy Resolution Colon …”

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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“…Liu et al [ 45 ] F/71y Fullness Tail Distal pancreatectomy Resolution – 11. Emily et al [ 46 ] F/40y Belly pain Tail Distal pancreatectomy Resolution Colon …”

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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“…We discuss the imaging findings and diagnosis from multi-phase contrast-enhanced computed tomography (CT) and fluorodeoxyglucose ( 18 F-FDG) positron emission tomography (PET-CT), as well as the patient's treatment and follow-up. While RDD can involve the GI system, such cases are still infrequent in the literature (4)(5)(6)(7)(8)(9). To the best of our knowledge, this is the first report of RDD affecting the sigmoid colon, and the utility of PET/CT in such a case has not been previously described.…”

Section: Introductionmentioning

confidence: 88%

Case report: A rare case of isolated sigmoid Rosai-Dorfman disease on contrast-enhanced CT and 18F-FDG PET/CT

Qi,

Luo,

Shao

et al. 2024

Front. Med.

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Rosai-Dorfman disease (RDD) is an uncommon histiocytic disorder that occurs in nodal and/or extranodal sites. Extranodal RDD exhibits a wide range of clinical and radiological presentations, frequently leading to misdiagnoses. Involvement of the gastrointestinal (GI) system is uncommon, accounting for less than 1% of the reported cases. Here we present a case of a 54-year-old male who complained of abdominal distention and was diagnosed with RDD affecting the sigmoid colon, manifesting as a sigmoid mass. The patient had a past medical history of liver transplantation due to hepatocellular carcinoma (HC). This report details the multiphase contrast-enhanced computed tomography (CT) and fluorodeoxyglucose (18F-FDG) positron emission tomography (PET-CT) imaging findings of RDD involving the sigmoid colon without lymphadenopathy, and a review of the relevant literature is provided.

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“…As such, the presentation of RDD is variable and may include bowel obstruction, CNS symptoms, or oropharyngeal symptoms. [1][2][3][4] Because little is understood about the pathogenesis of the disease, and the variable morbidity, the treatment strategies are varied and include resection, steroids, chemotherapy, and radiation therapy.…”

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confidence: 99%

“…1 Involvement of the gastrointestinal tract is exceedingly rare with only 20 documented cases and only 3 cases involving the rectum. 2,3 Here, we present 2 cases of rectal RDD with attention paid to the diagnostic and technical challenges. We present a video of a robotic low-anterior resection.…”

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confidence: 99%

Rosai-Dorfman Disease of the Rectum: Newly Identified Genetic Point Mutations and Robotic Resection

Lambdin

Phillips

Kima³

et al. 2022

The American Surgeon™

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Rosai-Dorfman disease (RDD) is a rare disease characterized by histiocytic proliferation which typically presents as massive, painless, cervical lymphadenopathy in children or young adults. GI involvement is exceedingly rare with only 20 documented cases to date. Of those 20 cases, only 3 cases have involved the rectum. Here, we present 2 cases of rectal RDD with attention paid to the diagnostic and technical challenges presented by this disease. When presenting as a perirectal mass, RDD can be mistaken for other lesions to include malignancy, leading to surgical removal. We present a video of a robotic low-anterior resection with intracorporeal anastomosis in order to remove a pelvic mass involving the rectum, initially considered to be a stromal tumor. In addition, we describe a copy number variation in AKT and 3 point mutations detected by next generation sequencing, which had not been previously reported in association with this disease.

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Synchronous Colon and Pancreatic Rosai-Dorfman Disease

Cited by 5 publications

References 14 publications

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

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

Case report: A rare case of isolated sigmoid Rosai-Dorfman disease on contrast-enhanced CT and 18F-FDG PET/CT

Rosai-Dorfman Disease of the Rectum: Newly Identified Genetic Point Mutations and Robotic Resection

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