A typical case of resected pancreatic hamartoma: a case report and literature review on imaging and pathology

Katayama, Hokahiro; Azuma, Kazufumi; Kono, Kenji; Murakami, Makoto; Hirono, Yasuo; Hatta, Satomi; Imamura, Yoshiaki; Goi, Takanori

doi:10.1186/s40792-020-00869-y

Cited by 12 publications

(17 citation statements)

References 26 publications

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“…Many of these reports describe smaller tumors that are found incidentally in asymptomatic patients, lesions that were resected due to concern for a malignant process of the pancreas or retroperitoneum, or tumors in patients with vague abdominal complaints likely due to other causes. The number of recent cases being reported has increased, likely due to the advancement in imaging quality and frequency of use (4). All tumors reported in the literature were small, averaging approximately 2-4 cm in diameter, with some patients presenting with multifocal lesions (2, 4, 6-8).…”

Section: Discussionmentioning

confidence: 99%

“…Very few cases of pancreatic lipomatous tumors with distinct components of pancreatic hamartoma have been described. Reports have suggested radiographic and pathologic features that are suggestive of both pancreatic hamartomas and pancreatic lipomatous hamartomas, such as no dilatation of the main pancreatic duct, well-de ned slight hyperintensity or iso-intensity on T2 weighted imaging, and isointensity on diffuse weighted imaging, with obvious progressive enhancement (2,4). Despite this, these tumors are notoriously di cult to diagnose on imaging and biopsy alone, as they can often appear similar to retroperitoneal liposarcomas and other benign or malignant tumors.…”

Section: Introductionmentioning

confidence: 99%

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A Massive Retroperitoneal Tumor – An Unusual Presentation of a Rare Primary Pancreatic Neoplasm

Clegg

Hammond

Schmocker

et al. 2022

Preprint

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Background:The differential diagnosis for retroperitoneal masses is broad, and includes pancreatic lipomatous hamartoma, a rare but benign disease process. Hamartoma describes outgrowth of tissue native to the organ in which it occurs and is considered a malformation rather than a true neoplasm. Pancreatic hamartomas consist of less than 1% of all hamartomas, and few cases of pancreatic lipomatous tumors with distinct components of pancreatic hamartoma have been described. These tumors are difficult to diagnose on imaging and biopsy alone and can appear malignant with potential for significant mass effect on surrounding organs, leading to surgical intervention for definitive diagnosis. Case Presentation:A 57-year-old male presented with right upper quadrant pain radiating to his left side and back. Ultrasound demonstrated cholelithiasis and an incidentally discovered large retroperitoneal mass. Computed tomography (CT) imaging demonstrated a 14.4 x 4.6 x 16 cm heterogenous, fatty-appearing retroperitoneal mass displacing the colon and small bowel, while abutting the aorta and inferior vena cava (IVC) with no obvious invasion of surrounding structures. He underwent laparoscopic cholecystectomy with biopsy of the mass. Biopsy demonstrated benign adipose tissue and no evidence of malignancy, and his care was transferred to our facility. His postoperative course was complicated by acute congestive heart failure that ultimately delayed definitive surgical intervention for nine months. Progressive growth of the mass was noted on serial imaging as his cardiopulmonary status was optimized. Preoperative CT revealed a 20.2 x 28.2 x 14.1 cm with worsened mass effect on surrounding structures.The patient underwent open resection of the retroperitoneal mass with en bloc resection of the third and fourth portions of the duodenum and the uncinate process of the pancreas. The final pathologic diagnosis was benign lipomatous proliferation with features most consistent with pancreatic lipomatous hamartoma (PLH), with specimen weight of 3810g and overall dimensions of 35 x 27 x 14 cm.Conclusions:PLH is an extremely rare benign tumor, which is notoriously difficult to diagnose with imaging and biopsy alone. This case highlights the difficulty delineating benign from malignant tumors of the pancreas and retroperitoneum.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A Massive Retroperitoneal Tumor – An Unusual Presentation of a Rare Primary Pancreatic Neoplasm

Clegg

Hammond

Schmocker

et al. 2022

Preprint

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“…Pancreatic hamartoma was first reported in 1977 ( 2 ), and cases have occasionally been reported since. A search of the PubMed database with the keywords “pancreatic hamartoma” rendered 46 cases, including the present case, of which 4 were children and 42 were adults ( 1 - 26 ) ( Table 2 ). The mean age of the patients was 52.5 years (range: 0.6 to 78), and the men:women ratio was 24:22, with most diagnoses made either by chance or because of abdominal pain.…”

Section: Discussionmentioning

confidence: 99%

Pancreatic Hamartoma Difficult to Diagnose Preoperatively

et al. 2021

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Abdominal ultrasonography in a 70-year-old woman showed a hypoechoic mass, 14 mm in diameter, in the pancreatic body. Computed tomography showed a mass with contrast effect in the pancreatic body. Test results for endocrine factors or tumor markers were normal. The initial consideration was nonfunctional pancreatic neuroendocrine tumor. Over 8 years of monitoring, the tumor diameter increased to 18 mm, until pancreatic tumor enucleation was performed. The postoperative diagnosis was pancreatic hamartoma, a rare type of benign pancreatic tumor. The preoperative diagnosis of pancreatic hamartoma is difficult, but consideration must be given to the possibility of hamartoma when encountering pancreatic tumors.

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“…2 Pancreatic hamartoma is an extremely rare entity, with only 41 reported cases to date, including our case. 3 Here, we report a case of a pancreatic hamartoma whose radiological findings mimicked those of a neuroendocrine tumor. The median age of patients was 51.9 years (range, 34 weeks to 78 years), and there was no significant difference in the numbers of male and female (male-to-female ratio 1.4:1.0).…”

mentioning

confidence: 89%

“…The mean size of the pancreatic hamartomas was 4.3 cm (0.9-19.0 cm), and they were predominantly located in the head of the pancreas (66%). 3 Histopathological findings reveal distorted architecture of pancreatic hamartoma in the normal pancreas due to which it is considered as a malformative lesions. 4 Pancreatic hamartoma presents mature ductal and acinar structures within abundant fibrous stroma.…”

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confidence: 99%