Tumors of the ampulla of Vater--local or radical resection?

Duodenal ampulla is a complex anatomical and histological site and a tumor may arise from one of three types of epithelium: duodenal mucosa, pancreatic duct and distal common bile duct. Neoplasia from each of these locations may exhibit different growth models and though it is difficult to establish their origin before operation, surgical treatment for all resectable tumors is essentially alike. The outcome of these tumors is vitally influenced by the type of epithelium from which they derive. Benign adenomas appear to be a frequent precursor of carcinoma of the ampulla of Vater, therefore, a local resection can lead to an under treated early cancer which would have benefited from a radical excision, with a much better long term result. We consider acceptable to perform an ampullectomy whenever is possible to safely state the benignity of the tumor or when a major procedure is hazardous.     

Resection of Tumors of the Neck of the Pancreas with Venous Invasion: the “Whipple at the Splenic Artery (WATSA)” Procedure

Introduction  

Tumors of the neck of the pancreas may involve the superior mesenteric and portal veins as well as the termination of the splenic vein. This presents a difficult problem since the pancreas cannot be transected through the neck as is standard in a Whipple procedure. Here, we present our method of resecting such tumors, which we term “Whipple at the Splenic Artery (WATSA)”.     

Is the New Classification of Neuroendocrine Pancreatic Tumors of Clinical Help?

A new concept of classifying neuroendocrine pancreatic tumors based on clinicopathologic patterns was summarized recently. To evaluate the clinical reliability and prognostic specificity of this classification system, 100 neuroendocrine pancreatic tumors were retrospectively categorized as “benign,”“uncertain,” and “malignant” based on tumor risk factors (size, local invasion and angioinvasion, cell atypia, metastases) and were followed for disease recurrence and progression. Altogether, 71 functioning tumors (insulinoma, gastrinoma, glucagonoma, enterochromaffin-like (ECL)oma, somatostatinoma) and 29 nonfunctioning neuroendocrine pancreatic tumors (NETs) were studied. NETs had an increased risk of malignancy (p < 0.05). Tumor size, gross invasion, and metastases correlated significantly with tumor behavior and allowed us to distinguish between “benign” and “malignant” tumors. About 89% of the tumors ≤ 20 mm were “benign,” whereas 71% > 20 mm were “malignant” (p < 0.05). In patients with “benign” and “uncertain” neuroendocrine pancreatic tumors, neither recurrence nor progression of disease was seen. About 41% of the patients with “malignant” tumors died of the disease. The 5-year estimated cumulative survival of those with “benign” and “uncertain” tumors was 100% and 52 ± 10% for those with “malignant” tumors (p < 0.05). Histomorphologic details classifying the behavior of an “uncertain” tumor are known only after initial treatment and definitive histopathologic investigation. Thus this information is of limited clinical help for treatment strategies.     

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

The Ewing’s family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7–70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1–168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.     

Diagnostically Challenging “Fatty” Retroperitoneal Tumors

A variety of benign and malignant retroperitoneal mesenchymal lesions may have a component of adipose tissue, including entities such as lipoma, myolipoma, angiomyolipoma, solitary fibrous tumor, genital stromal tumors, and well-differentiated/dedifferentiated liposarcoma. Although definitive diagnosis is usually straightforward on the complete resection specimen, it is often more difficult to workup these lesions on small biopsy samples. This review focuses on challenging diagnostic scenarios of retroperitoneal lesions with a “fatty” component and provides major differential diagnoses for commonly encountered morphologic patterns, clinicopathologic features of the various entities, and strategy for use of ancillary techniques, such as immunohistochemistry and cytogenetic studies.     

Do Orthopaedic Oncologists Agree on the Diagnosis and Treatment of Cartilage Tumors of the Appendicular Skeleton?

Background

Distinguishing a benign enchondroma from a low-grade chondrosarcoma is a common diagnostic challenge for orthopaedic oncologists. Low interrater agreement has been observed for the diagnosis of cartilaginous neoplasms among radiologists and pathologists, but, to our knowledge, no study has evaluated inter- and intraobserver agreement among orthopaedic oncologists grading these lesions using initial clinical and imaging information. Determining such agreement is important since it reflects the certainty in the diagnosis by orthopaedic oncologists. Agreement also is important as it will guide future treatment and prognosis, considering that there is no gold standard for diagnosis of these lesions.

Questions/Purposes

(1) to determine inter- and intraobserver agreement among a multinational panel of expert orthopaedic oncologists in diagnosing cartilaginous neoplasms based on their assessment of clinical symptoms and imaging at diagnosis. (2) To describe the most important clinical and imaging features that experts use during the initial diagnostic process. (3) To determine interobserver agreement for proposed initial treatment strategies for cartilaginous neoplasms by this panel of evaluators.

Methods

Thirty-nine patients with intramedullary cartilaginous neoplasms of the appendicular skeleton of various histopathologic grades were selected and classified as having benign, low-grade malignant, or intermediate- or high-grade malignant neoplasms by 10 experienced orthopaedic oncologists based on clinical and imaging information. Additionally, they chose the three most important clinical or imaging features for the diagnosis of these neoplasms, and they proposed a treatment strategy for each patient. The Kappa coefficient (κ) was used to determine inter- and intraobserver agreement.

Results

Inter- and intraobserver agreements were only fair to good, κ = 0.44(95% CI, 0.41–0.48) and κ = 0.62 (95% CI, 0.52–0.72), respectively. The three factors most frequently identified as helpful in making the diagnosis by our panel were cortical involvement in 65% of evaluations (253/390), neoplasm size in 51% (198/390), and pain in 50% (194/390). The interobserver agreement for the proposed initial treatment strategy after diagnosis was poor (κ = 0.21; 95% CI, 0.18–0.24).

Conclusions

This study showed barely fair interobserver and fair to good intraobserver agreement for grading of intramedullary cartilaginous neoplasms by orthopaedic oncologists using initial clinical and imaging findings. These results reflect the insufficient guidance interpreting clinical and imaging features, and the limitations of the systems we use today when making these diagnoses. In the same way, they generate concern for the implications that this may have on different treatment strategies and the future prognosis of our patients. Future studies should build on these observations and focus on clarifying our criteria of diagnosis so that treatment recommendations are standardized regardless of the treating institution or oncologist.

Level of Evidence

Level III, diagnostic study.