Pancreatic Desmoid Tumor Research Articles

Desmoid fibromatosis involving the pancreas: A retrospective case series with clinical, cytopathologic and radiologic correlation

Desmoid-type fibromatosis (desmoid tumors) which involve the pancreas is an infrequent diagnosis which clinically can mimic both neoplastic and non-neoplastic lesions of the pancreas. The cytologic features of loosely cohesive cytologically bland (myo)fibroblastic cells are non-specific, however the long fascicular growth pattern and the presence of β-catenin mutation with positive nuclear immunohistochemical staining or molecular testing allows for definitive diagnosis. While many previously reported desmoid tumors of the pancreas have been surgically resected, conservative management with a “watch and wait” approach is also an effective mode of management for these tumors. Herein, we report the largest case series of pancreatic desmoid tumors with clinical, cytopathologic, and radiologic correlation.

A Case of Pancreatic Desmoid Tumor Treated by Laparoscopic Distal Pancreatectomy

A Case of Pancreatic Desmoid Tumor Treated by Laparoscopic Distal Pancreatectomy

Robotic-Assisted Distal Pancreatectomy for a Pancreatic Desmoid Tumor: A Case Report and Review of the Literature

Introduction: Desmoid tumors are rare, indolent tumors of mesenchymal origin, with a potential for local invasion and adjacent organ involvement, without a metastatic potential. Pancreatic desmoid tumors are defined as tumors involving the pancreas parenchyma, sparing adjacent organs, and requiring a pancreatectomy for adequate surgical resection. Case presentation: We report a case of a lesser sac heterogeneous solid tumor involving the pancreatic tail in a 69-year-old man without a known genetic predisposition. Treatment consisted of a robotic-assisted distal pancreatectomy and splenectomy due to the intimate anatomical association of the mass to the pancreatic parenchyma and splenic vessels. The mass was found to be a desmoid-type fibromatosis on pathological assessment. Discussion: Surgical resection with adequate margins remains the gold curative standard for desmoid-type fibromatosis tumors of the abdomen and retroperitoneum. Pancreatic desmoid tumors tend to be sporadic, occur equally among genders, most commonly involving the distal body and tail of the pancreas, requiring a distal pancreatectomy for adequate surgical margins. Conclusion: We report in this article the twenty first case of a pancreatic desmoid tumor in the literature and the first report of a robotic-assisted resection of this tumor.

A Sporadic Desmoid Tumor: an Exceptional Pancreatic Cystic-Solid Mass.

Desmoid tumors are locally aggressive and non-metastatic neoplasms with a high rate of recurrence. Desmoid tumors of the pancreas are, however, very rare, and only a few cases have been reported in the literature. This paper reports an anecdotal case of a diffuse pancreatic desmoid tumor with the involvement of the pancreatic head, body, and-partially-tail. The patient underwent the Whipple procedure and subtotal pancreatectomy. Histopathological assessment showed that the tissues were partly positive for smooth muscle actin, but not for S100 or PanCK. The Ki67 index of the cells was only 1%. Unfortunately, the patient died on the 10th postoperative day due to massive upper gastrointestinal bleeding.

Pancreatic desmoid tumor in a 4-year-old male with hemihypertrophy

We report the first case of a pancreatic desmoid tumor detected during follow-up for hemihypertrophy in a 4-year-old boy. Hemihypertrophy is a rare disorder in which one side of the body grows more than the other, causing asymmetry, and well-known complications include embryonal tumors. However, there has been no report of desmoid tumors in patients with hemihypertrophy, and these tumors are rare and poorly characterized in the literature, especially the cystic variant. For this patient, the lesion was diagnosed as a desmoid tumor based on immunostaining positive for beta-catenin and mutation of the beta-catenin gene ( CTNNB1 ). This case suggests that desmoid tumors should be considered a possible etiology of pancreatic cystic lesions in patients with hemihypertrophy. • Desmoid tumors, especially cystic lesions, are rare and poorly characterized. • Desmoid tumors in patients with hemihypertrophy have never been reported. • Surgeons should consider desmoid tumors in cases of pancreatic cystic findings.

Ruptured pancreatic desmoid tumor presenting as hemorrhagic shock: a rare cause of acute abdomen

Ruptured pancreatic desmoid tumor presenting as hemorrhagic shock: a rare cause of acute abdomen

Isolated desmoid tumor of pancreatic tail with cyst formation diagnosed by beta-catenin immunostaining: a rare case report with review of literature.

Isolated pancreatic desmoid tumors with cyst formation are uncommon benign mesenchymal soft tissue tumors, characterized by the dense fibroblastic proliferations with abundant extra-cellular collagen matrix. Intra-abdominal desmoid tumor usually involve the mesentery and retroperitoneum and mostly occur in association of familial adenomatous polyposis or Gardner's syndrome. While desmoid tumors do not metastasize, their advancement can be life threatening due to aggressive local invasion, such as mesentery involvement. Isolated, sporadic pancreatic desmoid tumors have been considered anecdotal, with only 10 cases (cystic area in three cases) described in the literature. To our best of knowledge, this patient is fourth case report displaying cyst formation in desmoid tumor of pancreatic tail.We herein report a very unusual location of sporadic desmoid tumor involving the pancreatic tail with cystic area diagnosed by beta-catenin immunostaining. A 11-year-old male presented with painless lump in left hypochondrium of abdomen. The diagnosis of pancreatic adenocarcinoma was suspected preoperatively and the patient underwent a splenopancreatectomy. Histopathological examination revealed dense fibroblastic proliferation with occasional mitosis suggestive of mesenchymal tumor. The diagnosis of desmoid tumor was confirmed by positivity of beta-catenin immunohistochemical analysis. Conservative treatment was given postoperatively. No recurrence was observed after ten months of follow-up.Desmoid tumors are very rare in the tail of pancreas with cystic area and their diagnosis can be difficult, such as in our case where it presented as a solid-cystic lesion.

Management of Sporadic Pancreatic Desmoid Tumors

Management of Sporadic Pancreatic Desmoid Tumors