PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas.” (Hepatobiliary Pancreat Dis Int. 2006 Aug;5(3):454-8). Authors are Zhang H, Liang TB, Wang WL, et al., from the Department of Hepatobiliary Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, China. Solid-pseudopapillary tumor (SPT) of the pancreas is a rare exocrine pancreatic tumor. Despite the increasing recognition of the tumor in recent years, its pathogenesis and apparent therapeutic algorithm remain unclear. The study was designed to define the clinical, imaging, and pathologic features and to improve the diagnosis and treatment of this rare disease. The clinical, imaging, and pathologic findings of 9 SPT patients managed in the hospital between 2001 and 2005 were retrospectively analyzed, and related literatures were reviewed. SPT exhibits unique clinical and pathologic features and is readily diagnosed by its characteristic imaging and histological appearance. Surgical resection of the primary tumor and metastases is the treatment of choice. To access the full abstract of the article, click here.
Publications
On 28 July 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 388. The active ingredient is becatecarin for treatment of cancers of the biliary tree.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Granuloma faciale: Is it a new indication for pimecrolimus?” (J Dermatolog Treat. 2006;17(4):238-40). Authors are Eetam I, Ertekin B, Unal I et al., from the Department of Dermatology, Ege University Medical Faculty, Bornova-Izmir, Turkey. Granuloma faciale is a benign, chronic disease which is characterized by red-brown facial nodules and plaques. This quite rare disease is mostly seen in middle-aged males. The authors present a case of a 41-year-old female patient consulted in their department with a facial lesion of 4 years’ duration. A dermatological examination revealed a pink-brown plaque of 1×5 cm over the nasal dorsum extending to the left malar region. The routine hemogram and biochemical tests were normal, antinuclear antibody was negative. After histopathological examination of the biopsy material which was taken from the lesion the patient was diagnosed with granuloma faciale. To access the full abstract of the article, click here.
On 28 July 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation 387. The active ingredient is amikacin sulfate (liposomal) for treatment of pseudomonas aeruginosa lung infection in cystic fibrosis.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Mutations in SLC34A2 Cause Pulmonary Alveolar Microlithiasis and Are Possibly Associated with Testicular Microlithiasis.” (Am J Hum Genet. 2006 Oct;79(4):650-6). Authors are Corut A, Senyagit A, Ugur SA et al, from the Department of Molecular Biology and Genetics, Bogazici University, Istanbul, Turkey. Pulmonary alveolar microlithiasis, (PAM) is a rare disease characterized by the deposition of calcium phosphate microliths throughout the lungs. The authors first identified a PAM locus by homozygosity mapping to 4p15, then identified the gene responsible for the disease as SLC34A2 (the type IIb sodium-phosphate cotransporter gene), which is involved in phosphate homeostasis in several organs. Testicular microlithiasis (TM) is a disease that is more common than PAM. It is often associated with cancer and infertility. Since the gene identified is also expressed in testis, the authors searched for mutations in subjects with TM. In 2 of the 15 subjects with TM, two rare variants were identified, that are possibly associated with the condition. To access the full abstract of the article, click here.
On 28 July 2006, a new orphan medicinal product was registered into the community register of orphan medicinal products under the EU orphan designation number 386. The active ingredient is 4-[123I”>iodo-L-phenylalanine, indication: diagnosis of glioma.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Newborn infant with hemophagocytic lymphohistiocytosis and generalized skin eruptions.” (J Dermatol. 2006 Sep;33(9):628-31). Authors are Bay A, Calka O, Akdeniz N et al., from the Faculty of Medicine, Yuzuncu Yil University, Van, Turkey. Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from abnormal proliferation of histiocytes in tissues and organs. The incidence of HLH is 1:50 000-300 000. Cutaneous eruptions have been reported in 6-65% of the cases. The authors present an infant with prominent skin manifestations of HLH. On the 11th day of life, she was admitted to the hospital with complaint of a generalized rash that had started the previous day. The eruptions consisted of irregularly shaped maculopapular erythematous rash and purpura. Bone marrow aspiration on the 25th day of life revealed hemophagocytosis with increased macrophages and histiocytes, consistent with HLH. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Merkel cell carcinoma – a rarity in the urogenital tract.” (Onkologie. 2006 Jul;29(7):326-8. Epub 2006 Jul 3 ). Authors are Schwindl B, Meissner A, Giedl J еt al., from the Department of Urology, Klinikum Weiden, Germany. Merkel cell carcinoma – a rare, aggressive cancer of the skin integument – is being increasingly diagnosed but represents an absolute rarity in the urogenital tract. The authors report on a 70-year-old man who was referred to them with suspected testicular cancer. The pathology report revealed a metastasized Merkel cell carcinoma. The patient described is considered to be the first to develop testicular metastasis derived from Merkel cell carcinoma. Besides neuroendocrine and epithelial antigen tests, somatostatin receptor scintigraphy is a helpful diagnostic tool. To access the full abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Tenderness over the hyoid bone can indicate epiglottitis in adults” (J Am Board Fam Med. 2006 Sep-Oct;19(5):517-20). Author is Ehara H, from the Ehara Clinic, Tokyo, Japan. Adult acute epiglottitis is a rare but life-threatening disease caused by obstruction of the airway. The symptoms and signs of this disease may be nonspecific without apparent airway compromise. The author encountered 3 consecutive cases of adult patients with this disease in a single 5-month period in one physician’s office. In all cases, physical examination revealed tenderness of the anterior neck over the hyoid bone. These observations assisted the authors in identifying this rare disease quickly. They suggest that tenderness over the hyoid bone should raise suspicion of adult acute epiglottitis. To access the abstract of the article, click here.
PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Popliteal Sarcomas: Presentation, Prognosis and Limb Salvage” (Clin Orthop Relat Res. 2006 Aug 31). Authors are Pritsch T, Bickels J, Winberg T et al, from the Department of Orthopedic Oncology, Washington Cancer Institute, Washington Hospital Center, Washington, DC; The National Unit of Orthopedic Oncology, Tel-Aviv Sourasky Medical Center, Tel-Aviv, Israel; Georgetown University, Washington, DC; and the Pediatric and Surgery Branch, National Cancer Institute, Bethesda, USA. Soft tissue sarcomas of the popliteal fossa are rare diseases. The authors ascertained the mode of initial presentation, the rates of local recurrence and distant metastasis, and the morbidity and complications of limb salvage procedures by retrospective analyze of the data of 29 consecutive patients operated on between 1989 and 2003. Sixteen, five, and eight patients were diagnosed with high-, intermediate-, and low-grade tumors, respectively. Soft tissue sarcomas of the popliteal fossa have an atypical presentation. Limb salvage can be accomplished in most patients with low morbidity and good systemic and local control. To access the full abstract of the article, click here.