'Telangiectatic' transformation in soft tissue sarcomas. a clinicopathology analysis of an aggressive feature of high-grade sarcomas.

Background: 'Telangiectatic' change, which contains a large fluid hemorrhagic component, occurs in a variety of high-grade soft tissue sarcomas.

Methods: In a retrospective database review, we identified 20 consecutive patients (3%) with 'telangiectatic' change in soft tissue sarcomas.

Results: Tumors were located in the thigh (55%), shoulder (15%), calf (15%), upper arm (10%), and buttock in one patient.

Paratesticular leiomyosarcoma in an elderly man.

Soft tissue tumors, both benign and malignant, of the testes are relatively uncommon. Primary leiomyosarcoma of the testis is rare, and its clinical behavior is difficult to predict. We report a case of paratesticular leiomyosarcoma in a 71-year-old man.

Diagnosis of gastrointestinal stromal tumors: A consensus approach.

As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GISTs), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention by pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues.

Diagnosis of gastrointestinal stromal tumors: a consensus approach.

As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GIST), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor STI-571, these tumors have become the focus of considerable attention among pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a source of confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues.

Diagnosis of primary bone tumors with image-guided percutaneous biopsy: experience with 110 tumors.

Purpose: To determine the diagnostic accuracy of image-guided percutaneous biopsy in 110 primary bone tumors of varying internal compositions.

Materials And Methods: One hundred ten consecutive patients with primary bone tumors underwent biopsy with computed tomography (CT) or fluoroscopy. Ninety-one patients underwent surgical follow-up and 19 received medical treatment and underwent subsequent imaging studies.

Osteosarcoma: a multidisciplinary approach to diagnosis and treatment.

The treatment of osteosarcoma requires a multidisciplinary approach involving the family physician, orthopedic oncologist, medical oncologist, radiologist and pathologist. Osteosarcoma is a mesenchymally derived, high-grade bone sarcoma. It is the third most common malignancy in children and adolescents.

Pedunculated peritoneal surface polyps in pseudomyxoma peritonei syndrome.

Aims: Pseudomyxoma peritonei syndrome is a rare disease that originates from an adenomatous lesion of the appendix that, from pressure, perforates to gain access to the free peritoneal cavity. The relative sparing of the small bowel surfaces allows for complete cytoreduction even though many kilograms of mucinous tumour exist at other sites within the abdomen and pelvis. The purpose of this study was to examine the mechanism whereby the small bowel remains free of gross tumour and peritoneal surface polyps form.

Molecular genetic analysis of appendiceal mucinous adenomas in identical twins, including one with pseudomyxoma peritonei.

Pseudomyxoma peritonei (PMP) is a clinical syndrome characterized by mucinous ascites and peritoneal lesions composed of histologically bland to low-grade adenomatous mucinous epithelium within pools of extracellular mucin, often with an associated mucinous adenoma of the appendix. There is evidence that the peritoneal lesions in PMP are clonally derived from the associated appendiceal adenoma. Little is known about the molecular genetic alterations or hereditary factors involved in the development of appendiceal mucinous tumors and PMP.

Survival after induction chemotherapy and surgical resection for high-grade soft tissue sarcoma. Is radiation necessary?

Background: Induction chemotherapy can produce dramatic necrosis in sarcomas-raising the question of whether or not radiation is necessary. This study reviews the clinical outcome of a subset of patients with high-grade extremity soft tissue sarcomas (STS) who were treated with induction chemotherapy and surgical resection but without radiation.

Methods: Nonmetastatic, large, high-grade STS of the pelvis and extremities were treated with intra-arterial cisplatin, adriamycin, and, after 1995, ifosfamide.

Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis.

Background: Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by disseminated intraperitoneal mucinous tumors, often with mucinous ascites. The term PMP has been applied historically as a pathologic diagnostic term to both benign and malignant mucinous neoplasms that produce abundant extracellular mucin, resulting in a variable and poorly predictable prognosis. A recent study reported a pathologic classification that separated patients into prognostically distinct groups, but the follow-up was relatively short.

Peritoneal carcinomatosis from an unknown primary site. Management of 15 patients.

Aims And Background: Peritoneal carcinomatosis from an unknown primary site is a rare and ill-defined entity. This work attempts to identify clinical and pathological features of patients with this disease and report the results of an aggressive combined treatment modality.

Methods: Retrospective analysis was performed of medical records of 15 patients with peritoneal carcinomatosis with no primary site identified at a single institution between 1989 and 2000.

Histopathologic analysis in 46 patients with pseudomyxoma peritonei syndrome: failure versus success with a second-look operation.

Pseudomyxoma peritonei syndrome is a disease characterized by mucinous ascites and mucinous tumor disseminated on peritoneal surfaces; the disease almost always originates from a perforated appendiceal epithelial tumor. Histopathologic assessment of aggressive versus noninvasive character of the mucinous tumor has been shown to have an impact on survival in patients treated with cytoreductive surgery and intraperitoneal chemotherapy. Out of a database of 312 patients having a complete cytoreduction for pseudomyxoma peritonei syndrome, 46 patients (24 male and 22 female) had at least one second-look surgery.

The percutaneous needle biopsy is safe and recommended in the diagnosis of musculoskeletal masses.

Background: The purpose of this study was to analyze the role of percutaneous core needle biopsy in the diagnosis of musculoskeletal sarcomas.

Methods: One hundred eighty-five biopsy procedures were performed on 161 musculoskeletal tissue masses suspected of being a sarcoma in 155 patients who underwent subsequent tumor resection. A percutaneous core needle biopsy was performed on all masses either in the clinic or under radiologic guidance.

Results of treatment of 33 patients with peritoneal mesothelioma.

Background: Peritoneal mesothelioma is a rare peritoneal malignancy, representing approximately one-third of all mesotheliomas. It is regarded as a universally fatal cancer with few treatment options.

Methods: Records of 33 patients with peritoneal mesothelioma were reviewed retrospectively.

Early localized peritoneal mesothelioma as an incidental finding at laparoscopy. Report of a case and implications regarding natural history of the disease.

Background: Peritoneal mesothelioma is regarded as a fatal disease that presents with progressive ascites in a relatively late stage of its natural history. To the authors' knowledge, prior published articles have not described the early manifestations of this cancer.

Methods: A 30-year-old asymptomatic woman underwent laparoscopy for an infertility workup.

Tall cell variant: an aggressive form of papillary thyroid carcinoma.

Twenty-four cases of the tall cell variant (TCV), a subset of papillary thyroid carcinoma, were identified in a group of 624 patients with thyroid cancer. All pathology specimens were reviewed, and each patient's carcinoma was categorized according to characteristics on presentation, local recurrence, distant metastases, follow-up, and tumor-related mortality. The TCV group was compared with a historical control group (Mazzaferri and Jhiang: 1355 patients).

Biopsy of musculoskeletal tumors. Current concepts.

Biopsy is a key step in the diagnosis of bone and soft tissue tumors. An inadequately performed biopsy may fail to allow proper diagnosis, have a negative impact on survival, and ultimately necessitate an amputation to accomplish adequate margins of resection. Poorly performed biopsy remains a common finding in patients with musculoskeletal tumors who are referred to orthopaedic oncology centers.

Epithelioid sarcoma: an immunohistochemical analysis of 112 classical and variant cases and a discussion of the differential diagnosis.

Epithelioid sarcoma (ES) is a distinctive soft tissue neoplasm with a predilection for the distal extremities of young adults. This tumor typically contains nodular aggregates of epithelioid and spindle cells with zonal necrosis. The neoplastic cells are generally reported to coexpress keratin and vimentin and are often stated to be positive for CD34.

The role and biology of cryosurgery in the treatment of bone tumors. A review.

The application of liquid nitrogen as a local adjuvant to curettage in the treatment of bone tumors was introduced three decades ago. This technique, termed cryosurgery, was shown to achieve excellent local control in a variety of benign-aggressive and malignant bone tumors. However, early reports showed that cryosurgery was associated with a significant injury to the adjacent rim of bone and soft-tissue, resulting in high rates of fractures and infections.

Appendiceal mucocele. Contraindication to laparoscopic appendectomy.

Indications and contraindications to laparoscopic surgery continue to be refined. Laparoscopic appendectomy for acute appendicitis is frequently selected by patients and surgeons, and clinical studies show it to be a reasonable alternative. In this case study, laparoscopic surgery was used to resect an appendiceal mucocele caused by a nonperforated mucinous adenocarcinoma.

Thyroid papillary carcinoma of columnar cell type: a clinicopathologic study of 16 cases.

Background: Thyroid papillary carcinoma of columnar cell type is considered an uncommon histologic subtype of papillary carcinoma characterized by its morphologic features and purportedly aggressive biologic course.

Methods: Sixteen cases of thyroid papillary carcinoma of columnar cell type were identified from the Endocrine Tumor Registry at the Armed Forces Institute of Pathology and the Washington Hospital Center. Clinical records and follow-up were available in all cases.

Pulmonary metastases in pseudomyxoma peritonei syndrome.

Background: Pseudomyxoma peritonei (PMP) is a rare disease arising from a mucinous cystadenoma of appendiceal origin. The syndrome has been characterized by progressive growth of mucinous tumors, tense mucinous ascites, and ultimately death. Abdominal and pelvic recurrence after resection of intraperitoneal disease occurs in all patients unless adjunctive measures are taken.

The morphologic spectrum of ovarian metastases of appendiceal adenocarcinomas: a clinicopathologic and immunohistochemical analysis of tumors often misinterpreted as primary ovarian tumors or metastatic tumors from other gastrointestinal sites.

Twenty cases of ovarian metastases derived from appendiceal adenocarcinomas were analyzed. The most common presentation was a pelvic mass. The appendiceal and ovarian tumors were diagnosed concurrently in 15 cases; in the remaining five, the ovarian tumors were diagnosed before the appendiceal tumor.