Abstract Background Synovial sarcoma is a malignant tumor that constitutes up to 10% of all soft-tissue sarcomas. The most frequent metastatic sites of synovial sarcoma are the lungs, lymph nodes, and bone, whereas pancreatic metastasis is extremely rare. Here, we report a case of pancreatic metastasis of synovial sarcoma. Case presentation Nine years before presentation, a 31-year-old woman underwent extensive resection of the primary tumor after chemotherapy for left upper extremity synovial sarcoma. Six months before presentation, interscapulothoracic amputation was performed for an enlarged mass in the left upper extremity; the patient was treated with pazopanib. Three months before presentation, chest computed tomography showed multiple lung metastases; during subsequent follow-up, abdominal computed tomography revealed a pancreatic metastasis of synovial sarcoma. The doubling time of the pancreatic tumor was 14 days, and it grew rapidly. Furthermore, treatment-resistant pancreatitis symptoms were detected; thus, we performed distal pancreatectomy and administered one course of a 70% dose of trabectedin. However, the patient died of rapid progression of lung metastasis and respiratory failure within 2 months after surgery. Conclusions Pancreatectomy may be carefully performed in cases of isolated pancreatic metastasis. However, the presence of other distant extrapancreatic metastases (e.g., uncontrolled lung metastases) may rule out pancreatectomy treatment. 

/pdf/early-progression-of-a-pancreatic-metastasis-of-synovial-fchghl1v.pdf

Early progression of a pancreatic metastasis of synovial sarcoma after pancreatectomy

Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease. In addition to pathology, molecular analysis is pivotal in differential diagnosis, especially with malignant melanoma. A key aspect in identifying clear cell sarcoma is specific genetic alterations, notably the translocation of t(12;22) (q13;q13), a diagnostic hallmark of this sarcoma subtype, which is absent in malignant melanoma. Treatment of primary clear cell sarcoma of the pancreas is the same as that for adenocarcinoma. 

Metastatic clear cell sarcoma of the pancreas: A sporadic cancer

Synovial sarcoma usually presents in the lower extremities and metastasizes to the lungs; however, unusual patterns of recurrence can occur. For patients with recurrent synovial sarcoma to a proximal peripancreatic lymph node, a pancreaticoduodenectomy or Whipple procedure is the best option for a cure. Lymph node metastasis from synovial sarcoma is exceptionally rare, and data guiding the use of the Whipple procedure for curative resection of metastatic synovial sarcoma are even more sparse. In this report, we describe the management of a patient with metastatic synovial sarcoma to a proximal peripancreatic lymph node with a pancreaticoduodenectomy.

/pdf/first-recurrence-of-synovial-sarcoma-presenting-with-8bw3umen.pdf

First Recurrence of Synovial Sarcoma Presenting With Solitary Pancreatic Mass

Synovial sarcoma is a mesenchymal spindle cell tumor. Primary pancreatic sarcomas are extremely rare. The present study describes a rare case of synovial sarcoma in the head of the pancreas. A 35-year-old male presented with left upper quadrant abdominal pain. An endoscopic ultrasound examination revealed a complex solid-cystic lesion in the pancreatic head. He had undergone pancreaticoduodenectomy (Whipple procedure). A histological examination yielded negative results for AE1/AE3, CD10, S100, CD34, desmin, smooth muscle actin, β-catenin, CD117, HMB45, chromogranin and synaptophysin. However, the results were positive for TLEI and vimentin, which is consistent with synovial sarcoma. Synovial sarcoma is a soft tissue malignant tumor. Primary pancreatic sarcomas frequently present as large, high-grade tumors in the pancreatic head. Histologically, there are several types of synovial sarcoma, such as monophasic, biphasic and poorly differentiated. A histological examination is necessary for the diagnosis as the imaging findings are not specifically suggestive of synovial sarcoma. The preferred course of treatment is complete resection with wide margins, followed by adjuvant chemotherapy and/or radiotherapy. Primary mesenchymal tumors of the pancreas are extremely uncommon. As a result, a diagnosis requires careful evaluation. Surgical resection is the main modality of treatment.

/pdf/primary-pancreatic-synovial-sarcoma-report-of-a-rare-case-1hp9hdy2.pdf

Primary pancreatic synovial sarcoma: Report of a rare case and review of the literature

Pancreatic metastases are rare, accounting for 2%–3% of pancreatic tumors. The pancreas represents an unusual metastatic site of synovial sarcoma (SS) outside the usual localizations (regional node...

https://journals.sagepub.com/doi/pdf/10.1177/2036361320983691

The management of pancreatic metastasis from synovial sarcoma of the soft tissue: A case report.

Neurofibromatosis type 1 is a genetic disease characterized by changes in skin pigmentation and the growth of tumors along nerves in the skin, brain, and other parts of the body. Neurofibromas are the most common benign tumours of Neurofibromatosis type 1 developing at any point along a nerve with a neuro-cutanous tropism. This genetic affection is an autosomal dominant disorder with complete penetrance and an important phenotypic variability. Disease manifestations occur at the childhood mostly before five years. Nevertheless, many de novo cases remain undiagnosed well into adult life. Neurofibroma that occur in the breast and constitute a revealing signs of the disease at adulthood are extremely rare.
We report a case of a 30-year-old woman presenting a neurofibroma of the breast revealing Neurofibromatosis type 1.

http://www.issr-journals.org/links/papers.php?journal=ijisr&application=pdf&article=IJISR-17-182-03

Neurofibroma of the breast revealing a Von Recklingenhausen disease: a case report

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The management of pancreatic metastasis from synovial sarcoma of the soft tissue: A case report.

Neurofibroma of the breast revealing a Von Recklingenhausen disease: a case report