Histiocytic neoplasms in the era of personalized genomic medicine.

PURPOSE OF REVIEW: Since the discovery of B-Raf proto-oncogene (BRAF) V600E mutations in histiocytic neoplasms, diverse kinase alterations have been uncovered in BRAF V600E-wildtype histiocytoses. The purpose of this review is to outline recent molecular advances in histiocytic neoplasms and discuss their impact on the pathogenesis and treatment of these disorders. RECENT FINDINGS: Activating kinase alterations discovered in BRAF V600E-wildtype Langerhans (LCH) and non-Langerhans cell histiocytoses (non-LCH) result in constitutive activation of the mitogen-activated protein kinase and/or phosphoinositide 3-kinases-Akt murine thymoma pathways. These kinase alterations include activating mutations in A-Raf proto-oncogene, mitogen-activated protein kinase kinase 1, neuroblastoma rat sarcoma viral oncogene homolog, Kirsten rat sarcoma viral oncogene homolog, and phosphatidylinositol-4,5-bisphosphate 3 kinase, catalytic subunit α kinases in LCH and non-LCH; BRAF, anaplastic lymphoma receptor tyrosine kinase, and neurotrophic tyrosine kinase, receptor type 1 fusions, as well as the Ets variant 3-nuclear receptor coactivator 2 fusion in non-LCH; and mutations in the mitogen-activated protein kinase kinase kinase 1 and Harvey rat sarcoma viral oncogene homolog kinases in LCH and histiocytic sarcoma, respectively. These discoveries have refined the understanding of the histiocytoses as clonal, myeloid neoplasms driven by constitutive mitogen-activated protein kinase signaling and identified molecular therapeutic targets with promising clinical responses to rapidly accelerated fibrosarcoma and mitogen-activated protein kinase kinase inhibition. SUMMARY: Genomic analyses over the last 6 years have identified targetable kinase alterations in BRAF V600E-wildtype histiocytic neoplasms. However, despite this progress, the molecular pathogenesis and therapeutic responsiveness of non-BRAF V600E kinase alterations are still poorly defined in these disorders.

Primary spermatic cord tumors: disease characteristics, prognostic factors, and treatment outcomes.

INTRODUCTION: Experience with management of spermatic cord tumors (SCTs) is uncommon. We utilized a large population-based cancer registry to characterize the demographic, pathological, treatment characteristics, and outcomes of SCTs. MATERIAL AND METHODS: The Surveillance, Epidemiology, and End Results database (1973-2007) was queried. RESULTS: From the database, 362 patients were identified with SCT. The annual incidence of SCT was 0.3 cases per million and did not change over time. The most common histologic types were liposarcoma (46%), leiomyosarcoma (20%), histiocytoma (13%), and rhabdomyosarcoma (9%). The median age of diagnosis for rhabdomyosarcomas was (26.3 y), whereas for other SCTs, it was (64.7 y) (P<0.001). On multivariate analysis, a worse outcome was observed with undifferentiated tumor grade, distant disease, positive lymph nodes, and leiomyosarcoma or histiocytoma cell histology. CONCLUSION: We describe the largest cohort of SCT studied to date. Liposarcoma was most common, while leiomyosarcoma and histiocytoma histologic subtypes were observed to be the most aggressive. Multivariate analysis revealed that tumor grade, stage, histologic type, and lymph node involvement were independently predictive of prognosis.

Strategy of surgical treatment of giant presacral tumor - a case report.

Presacral tumors are uncommon lesions that can be difficult to diagnose because of their nonspecific presenting signs and symptoms. Surgery is the mainstay of treatment as it establishes the diagnosis and prevents the adverse consequences associated with malignant degeneration and secondary bacterial infection. Large, highly vascularised pelvic tumors may pose intraoperative difficulties as bleeding and intraoperative tumor perforation. Cross-sectional imaging is essential in evaluating these lesions to determine the optimal surgical approach and the extent of resection. We emphasize a multidisciplinary expert individualized approach. We report a case of a presacral giant gastrointestinal tumor initially considered as unresectable but further on successfully managed by preoperative vascular embolization followed by resection via abdomino-perineal approach.

Canine histiocytic diseases.

Canine histiocytic diseases are an emerging spectrum of diseases characterized by proliferations of histiocytic cells. Nonneoplastic histiocytic disease (reactive histiocytosis, comprising cutaneous and systemic histiocytosis) is uncommon. Neoplastic histiocytic diseases include cutaneous histiocytoma, which is a benign histiocytic tumor, and localized and disseminated histiocytic sarcoma (previously known as malignant histiocytosis), which are malignant diseases. The differentiation of histiocytic diseases can be challenging. This article outlines the characteristics of each disease entity and details the clinicopathologic, histologic, immunohistochemical, prognostic, and therapeutic differences among them.

Acute haemorrhage in patients with advanced head and neck cancer: value of endovascular therapy as palliative treatment option.

AIMS: Acute or subacute haemorrhage is one of the most frightening complications in patients suffering from advanced head and neck cancer. Few articles report experience with superselective endovascular therapy for this purpose. Is endovascular therapy underestimated in the field of palliative head and neck cancer therapy? This study set out to investigate this question. PATIENTS AND METHODS: A review was undertaken of the clinical courses of seven patients (six men, one woman) suffering from incurable, advanced head and neck cancer (four pharyngeal, two laryngeal, one neck) and treated with superselective endovascular strategies as an emergency procedure for acute bleeding. RESULTS: All patients were successfully treated without evidence of neurological complication. Patients reached a median survival of 20 weeks (range eight-168 weeks). Following endovascular treatment all patients were discharged from the hospital within several days. Three patients survived almost free of symptoms for several weeks and were able to stay at home with their families until their death. CONCLUSION: We conclude that in the field of palliative care, superselective endovascular therapy deserves to be considered alongside standard treatment options for the management of acute haemorrhage from advanced head and neck cancer.