Prevalence of left-sided melanomas in an Irish population.

BACKGROUND: A predominance of melanomas on the left side of the body has recently been described. No associations between tumour laterality and gender, age or anatomical site have been identified. AIM: The aim of this study was to investigate the prevalence of left-sided melanomas in an Irish population and to examine potential associations with various patient and tumour characteristics. METHODS: A retrospective chart review of patients with cutaneous melanoma who were treated over a 10-year period was carried out. Lateral distribution of melanoma on either side of the body was compared using χ(2) analysis and evaluated by gender, age group, anatomic location, histologic subtype and Breslow depth. RESULTS: More melanomas occurred on the left side (57%, P = 0.015), and this finding was particularly significant in females. For both genders combined, there were no statistically significant differences in laterality by age group, anatomic location, type of melanoma and Breslow depth. There were significantly more superficial spreading melanomas on the left side in both men and women. CONCLUSIONS: This study demonstrates a predominance of left-sided melanomas in Irish patients. While a number of demographic and molecular associations have been proposed, further research is required to fully explain this phenomenon.

Malignant melanoma and breast carcinoma: a bidirectional correlation.

BACKGROUND: Epidemiologic and genetic studies have suggested a bidirectional association between breast carcinoma (BC) and malignant melanoma (MM). OBSERVATION: We present a series of patients with MM and BC detected in our department within a span of 6 months, raising concerns for the high associations between the two malignancies. This led us to match the concordance of the two tumours in the National Irish Cancer Registry. CONCLUSION: The national figures provide evidence of a link between BC and MM. We recommend increased awareness among clinicians leading to more detailed surveillance of both second primary tumours. All MM patients with a family history of BC should be referred to a breast clinic. Women above the age of 40 with MM should undergo annual mammography and those less than 40 may be better evaluated with a breast MRI. All breast cancer patients should be made aware of the significance of changing moles and those with suspicious lesions referred to a dermatologist for evaluation.

Long-term results of Taiwan Pediatric Oncology Group studies 1997 and 2002 for childhood acute lymphoblastic leukemia.

The long-term outcome of 1390 children with acute lymphoblastic leukemia (ALL), treated in two successive clinical trials (Taiwan Pediatric Oncology Group (TPOG)-ALL-97 and TPOG-ALL-2002) between 1997 and 2007, is reported. The event-free survival improved significantly (P=0.0004) over this period, 69.3+/-1.9% in 1997-2001 to 77.4+/-1.7% in 2002-2007. A randomized trial in TPOG-97 testing L-asparaginase versus epidoxorubicin in combination with vincristine and prednisolone for remission induction in standard-risk (SR; low-risk) patients yielded similar outcomes. Another randomized trial, in TPOG-2002, showed that for SR patients, two reinduction courses did not improve long-term outcome over one course. Decreasing use of prophylactic cranial irradiation in the period 1997-2008 was not associated with increased rates of CNS relapse, prompting complete omission of prophylactic cranial irradiation from TPOG protocols, beginning in 2009. Decreased use of etoposide and cranial irradiation likely contributed to the low incidence of second cancers. High-risk B-lineage ALL, T-cell, CD10 negativity, t(9;22), infant, and higher leukocyte count were consistently adverse factors, whereas hyperdiploidy >50 was a consistently favorable factor. Higher leukocyte count and t(9;22) retained prognostic significance in both TPOG-97 and TPOG-2002 by multivariate analysis. Although long-term outcome in TPOG clinical trials is comparable with results being reported worldwide, the persistent strength of certain prognostic variables and the lower frequencies of favorable outcome predictors, such as ETV6-RUNX1 and hyperdiploidy >50, in Taiwanese children warrant renewed effort to cure a higher proportion of patients while preserving their quality of life.

Sweet's syndrome in association with common variable immunodeficiency.

Sweet's syndrome (SS), a rare reactive neutrophilic dermatosis, has been reported to occur in association with a variety of systemic disorders, categorized by von den Diesch into idiopathic, paraneoplastic, pregnancy and parainflammatory subgroups. The parainflammatory group has been well defined, and includes a wide spectrum of infectious triggers and disorders of immune dysregulation. To date, however, no cases of SS have been described in the context of common variable immunodeficiency (CVID). We report a case of paediatric-onset SS, previously reported as idiopathic, with a subsequent diagnosis of CVID.

Melanoma in organ transplant recipients: clinicopathological features and outcome in 100 cases.

Organ transplant recipients have a higher incidence of melanoma compared to the general population but the prognosis of this potentially fatal skin cancer in this group of patients has not yet been established. To address this, we undertook a multicenter retrospective analysis to assess outcome for 100 melanomas (91 posttransplant and 9 pretransplant) in 95 individuals. Data were collected in 14 specialist transplant dermatology clinics across Europe belonging to the Skin Care in Organ Transplant Patients, Europe (SCOPE) Network, and compared with age, sex, tumor thickness and ulceration status-matched controls from the American Joint Committee on Cancer (AJCC) melanoma database. Outcome for posttransplant melanoma was similar to that of the general population for T1 and T2 tumors (< or = 2 mm thickness); but was significantly worse for T3 and T4 tumors (> 2 mm thickness); all nine individuals with a pretransplant melanoma survived without disease recurrence following organ transplantation. These data have implications for both cutaneous surveillance in organ transplant recipients and management of transplant-associated melanoma.

Update on the pathogenesis of post-transplant skin cancer in renal transplant recipients.

Remarkable advances in the field of transplantation over the last several decades have benefited many thousands of patients. Five-year survival ranges from 90% for a live donor renal transplant to 85% for a cadaveric renal transplant. However, with this success come the complications of chronic immunosuppression. Lifelong immunosuppressive treatment for adequate graft function results in reduction of immunosurveillance, with increased risk of various cancers leading to substantial morbidity and mortality in these patients. This review discusses multifactorial intrinsic and extrinsic factors contributing to the pathogenesis of skin cancers in renal transplant recipients and reviews potential solutions.

Financial burden of national health insurance for treating patients with transfusion-dependent thalassemia in Taiwan.

The thalassemias are a heterogeneous group of inherited hypochromic anemias of varying severity. The mainstay of supportive treatment is regular blood transfusion accompanied by iron-chelating therapy. Hematopoietic stem cell transplantation (HSCT) provides an alternative option when curative therapy is considered. More than 400 patients in Taiwan have beta-thalassemia major or other transfusion-dependent thalassemias, and their treatment costs account for a considerable percentage of the National Health Insurance expenditure. In this report, we estimated the treatment costs of conventional therapy (regular blood transfusion accompanied by iron-chelating agents) and HSCT. The undiscounted medical cost of 20 years of follow-up (20 years from diagnosis) and the undiscounted total lifetime cost were NT$ 4 739 888 (NT$ means New Taiwan Dollars)/US$ 149 288 and NT$ 11 529 990/US$ 363 149, respectively, for patients undergoing conventional therapy, and NT$ 2 639 982/US$ 83 149 and NT$ 3 511 172/US$ 110 588, respectively, for those undergoing successful HSCT. Comparisons of treatment costs and other parameters between these two modalities can add to the information base on which policy is made by health authorities or clinicians.

The topical application of 2,3,7,8-tetrachlorodibenzo-p-dioxin lacks skin tumor-promoting potency but induces hepatic injury and tumor necrosis factor-alpha expression in ICR male mice.

One of the most toxic environmental pollutants known to man is 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). There is growing evidence that indicates TCDD is a potent tumor promoter in rat and mouse liver, as well as in mouse skin. The mouse skin carcinogenesis model has been used extensively to assess whether a chemical or physical agent carries a carcinogenic hazard to humans and to define the mechanism involved with the carcinogenic effects. We applied the mouse skin model to ICR male mice and the results showed that following the application of DMBA, repeated dorsal application of all doses of TCDD produced no papillomas. These findings imply that the ICR male mouse is an extremely insensitive strain as a TCDD-induced two-stage mouse skin carcinogenesis model. However, severe hepatic injuries and wasting syndrome were seen in mice treated topically with TCDD. Meanwhile, serum TNF-alpha levels increased during the experimental periods. Inflammatory cell infiltration, fatty liver, and nodule formation could be observed in damaged livers. Elevated hepatic EROD activity and urinary 8-epi-PGF2alpha were also observed in mice with short-term exposure of TCDD.

Liver resection for hepatocellular carcinoma in patients with end-stage renal failure.

BACKGROUND AND OBJECTIVES: Surgical resection remains the main option for curing hepatocellular carcinoma (HCC). However, liver resection in patients with end-stage renal disease (ESRD) is risky. The aim of this study is to clarify the role of liver resection for treating HCC in patients with ESRD. METHODS: A retrospective review was carried out on 468 patients who underwent liver resection for HCC between 1989 and 1999. The clinicopathological characteristics and operative results of 12 patients who had ESRD (ESRD group) were compared with those of the other 456 patients who did not have ESRD (non-ESRD group). In the ESRD group, heparin-free hemodialysis using the periodic saline-rinse method was performed during the perioperative period. RESULTS: The ESRD group had lower hemoglobin and a higher serum creatinine levels. Other patient background and tumor pathological characteristics were comparable between the two groups as well. The operative morbidity and mortality between the two groups were also similar. The 5-year disease-free survival rates for ESRD and non-ESRD groups were 35.0 and 34.2% (P = 0.31), respectively, while the 5-year actuarial survival rates were 67.8 and 53.3% (P = 0.54), respectively. CONCLUSION: With improving techniques and knowledge of dialysis, liver resection for HCC is justified in selected patients with ESRD.

Precocious puberty associated with growth hormone deficiency in a patient with craniopharyngioma: report of one case.

Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. Growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. Growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. Growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.

Histologic study of effects of radiation synovectomy with Rhenium-188 microsphere.

Rhenium-188 microsphere is a relatively new radiation synovectomy agent developed for the treatment of rheumatoid arthritis. It has been shown that the levels of unwanted extra-articular radiation are negligible with this agent. A histologic study was conducted to assess the effect of radiation synovectomy on synovium and articular cartilage after intra-articular injection of various doses of Re-188 microspheres into the knee joints of rabbits. Intra-articular injection of Re-188 microspheres into rabbit knee joints resulted in mild reactive inflammation and thrombotic occlusion of vessels which subsided rapidly. Sclerosis of subsynovium could be seen 12 weeks after injection. No evidence of damage to articular cartilage was noted. There was no significant difference in the articular pattern after injection of 0.3 or 0.6 mCi Re-188 microspheres. This study suggests that a treatment dose of Re-188 microspheres causes transient inflammation of synovium without any detectable damage to the articular cartilage of knee joint.

An appraisal of liver and portal vein resection for hepatocellular carcinoma with tumor thrombi extending to portal bifurcation.

BACKGROUND: The role of surgical resection for hepatocellular carcinoma with tumor thrombi involving the major portal vein is controversial because of a high operative risk and poor prognosis. Previously, a resection was performed only when the tumor thrombi were limited to the first branch of the portal vein without extension to the portal bifurcation. HYPOTHESIS: Concomitant liver and portal vein resection may be beneficial in patients with hepatocellular carcinoma with tumor thrombi extending to portal bifurcation. DESIGN: Retrospective review. SETTING: University hospital, tertiary referral center. PATIENTS: Among 368 patients with hepatocellular carcinoma who underwent a curative resection, portal vein involvement occurred in 112 patients. Fifteen of the 112 patients underwent a concomitant liver and portal vein resection owing to extension of tumor thrombi to the portal bifurcation (group 1). The remaining 97 patients did not need portal vein resection (group 2). INTERVENTION: Surgical indications, procedures, and results of pathological examination of resected specimens were assessed in patients in group 1. The clinicopathological characteristics, operative morbidity and mortality, and operative results were compared between the 2 groups. MAIN OUTCOME MEASURES: Disease-free and actuarial survival rates. RESULTS: Intramural tumor infiltration was found at the site of thrombi adhesion to the portal vein cuff in 11 of 15 patients in group 1. Owing to patient selection bias, patients in group 1 were significantly younger and had better liver function and greater resected liver weight. The operative time, postoperative hospitalization, operative blood loss, amount of blood transfusion, and operative morbidity and mortality did not differ significantly between the 2 groups. The 5-year disease-free survival rates of groups 1 and 2 were 21.6% and 20.4% (P =.19), respectively, while the actuarial survival rates were 26. 4% and 28.5% (P =.33), respectively. CONCLUSION: Liver resection with partial resection of the portal vein is justified in selected patients with hepatocellular carcinoma with tumor thrombi extending to portal bifurcation.

Histopathologic analysis of sentinel lymph nodes in breast carcinoma.

BACKGROUND: Intraoperative lymphatic mapping and identification of the first draining lymph node (the sentinel lymph node) may allow some patients with breast cancer to avoid the morbidity of formal axillary clearance. The aim of this study was to determine the accuracy of sentinel lymph node (SLN) biopsy in predicting axillary nodal involvement. METHODS: From August, 1998 until July, 1999, 41 patients with clinically node-negative breast cancer underwent SLN biopsy that was immediately followed by axillary lymph node dissection. If the SLN section was found free of metastasis by routine hematoxylin and eosin staining (H&E), then an additional four sections of the SLN were cut and examined for the presence of tumor cells by H&E staining (three sections) and by cytokeratin immunohistochemical staining (IHC) (one section). If the SLN had metastatic cells and the other remaining nonsentinel axillary lymph nodes were free of metastases by routine H&E staining, then an additional three sections of the nonsentinel axillary lymph nodes were cut and examined for the presence of tumor cells by H&E staining. RESULTS: The 41 patients had a mean of 2.2 sentinel (range, 1-7) and 14.6 nonsentinel (range, 5-32) lymph nodes excised per patient. Routine H&E staining identified 13 patients (31.7%) with SLN metastases and 28 patients (68.3%) with tumor-free SLNs. Applying IHC and the additional three sections stained with H&E to these tumor-free SLNs showed one additional patient with sentinel node metastasis. The conversion rate from being a sentinel node-negative patient to a sentinel node-positive patient was 3.6% (1/28). Overall, SLN metastases were detected in 14 (34.1%) of the 41 patients. The SLNs were negative in 27 patients (65.9%), two of whom had at least one positive nonsentinel lymph node each (7.4% "skip" metastasis). Biopsy of SLNs was 92.6% accurate in predicting the absence of nonsentinel nodal metastasis (p=0.001). CONCLUSIONS: Our results suggest that formal axillary lymph node dissection may need only be performed in SLN-positive patients. Nonetheless, further experience and refinement are needed to perfect this technique.

Detection of p53 mutations in sputum smears precedes diagnosis of non-small cell lung carcinoma.

Little progress has been made in reducing lung cancer mortality by applying conventional methods to early diagnosis and screening. Recent advances in molecular oncology, however, have provided tools which may be of use in this area. p53 gene mutation is the most common gene alteration in the development of lung cancer. Conventional cytologic analysis of sputum is an insensitive test for the diagnosis of lung cancer. In this study, we attempted to establish a polymerase chain reaction (PCR)-based assay for assessing the possibility of early detection of p53 mutation in archival Papanicolaou-stained cytologic sputum smears. Ten sputum smear slides were collected prior to clinical diagnosis from 10 lung cancer patients who had been confirmed to have p53 mutations in surgically resected lung tumors. We successfully obtained sufficient amounts of RNA from each sputum smear specimen for amplification of PCR and direct sequencing. Only one patient was found to have p53 mutation at codon 286; the other nine patients had wild type p53 genes. This result supports the possibility that detection of p53 mutations in cytologic sputum smears is an available strategy for the early diagnosis of lung cancer.

Hypohidrotic ectodermal dysplasia.

Hypohidrotic ectodermal dysplasia was first described by Thurnam in 1848. It is a rare, X-linked, recessive disorder characterized by anhidrosis or hypohidrosis, hypotrichosis, dental hypoplasia and characteristic facial features. Herein, we report a typical case of hypohidrotic ectodermal dysplasia. A 20-year-old male presented with the above symptoms at birth. When a family history was taken, it was discovered that his uncle (mother's brother) had the same characteristic facial features and hypotrichosis.

Lymph node revealing solution and traditional 10% buffered formaldehyde for detecting lymph nodes in colorectal carcinoma.

BACKGROUND: Cancer stage has been the most important factor in evaluating the prognosis and treatment of colorectal carcinoma, and the presence of lymph node metastasis in the resected specimen plays a key role in serial staging. We evaluated a newly proposed "lymph node revealing solution" (LNRS) that proponents claim is inexpensive, easy, rapid, and innocuous, and that could help to establish the stage of disease more accurately than buffered formaldehyde (formalin). Our study compared traditional 10% formalin and LNRS as second search solutions to determine whether LNRS is a more useful and practical solution than formalin. METHODS: Thirty randomly selected colorectal carcinomas from low anterior resection (LAR) were studied. The specimens were handled routinely on the first day. Then, the entire fat of odd-numbered cases was immersed in three times its volume of LNRS, and the entire fat of even-numbered cases was immersed in 10 times its volume of formalin overnight. The chi-squared test was used to evaluate the results. RESULTS: Of 145 cassettes in the second search by LNRS, seven had no lymph nodes. Of 107 cassettes in the second search by formalin, 18 had no lymph nodes. Totally, 792 lymph nodes were found with an average number of 26.4 per case and 1.53/cm (per centimeter of specimen) in all 30 cases. By the LNRS method, 138 lymph nodes were found in the 15 odd-numbered cases, and by the formalin method, 89 lymph nodes were found in the 15 even-numbered cases. We were able to adjust the stage upward in two cases by LNRS-fixation method. There were no changes in staging in the formalin-fixation method cases. CONCLUSIONS: LNRS showed no statistically significant difference from formalin in the numbers of total lymph nodes, positive lymph nodes, or minute (less than 1 mm) lymph nodes found. Nonetheless, we do believe that LNRS is a potential replacement solution for formalin due to its fast fixation, ease of searching, high identification rate and time-saving procedure.

Clinicopathologic analysis of renal and extrarenal angiomyolipomas: report of 44 cases.

BACKGROUND: Angiomyolipoma (AML) is a rare soft tissue tumor involving the kidneys, liver and other visceral organs. It is composed of smooth muscle cells, adipocytes and small- to medium-sized hyalinized vessels. The purpose of this study was to investigate the characteristic clinicopathologic manifestations of AML in different tissues. METHODS: We collected 37 renal, four hepatic, one lymph node, one subcutaneous and one uterine cervical AML to analyze the clinical, histologic, histochemical and immunohistochemical variations of AML in different tissues. RESULTS: Clinically, renal and hepatic AML were associated with tuberous sclerosis complex and predominate in women. There were several prominent pathologic differences between renal and extrarenal AML. First, none of the tumors were encapsulated except for the subcutaneous AML, which had a pseudocapsule. Secondly, the smooth muscle cells were usually epithelioid shaped in hepatic AML and occasionally in renal AML, but not in subcutaneous or uterine AML. Furthermore, hepatic AML characteristically showed extramedullary hematopoiesis and eosinophilic hyaline granules. Finally, only the tumor cells of renal, lymph node and hepatic AML had periodic acid-Schiff (PAS), PAS-D (after diastase digestion) positive granules and monoclonal mouse anti-human melanosome clone (HMB-45) immunoreactivity. CONCLUSIONS: By combining the clinical, histologic, histochemical and immunohistochemical features, some discrepancies between the renal and extrarenal AML were observed, such as female predominance, association with tuberous sclerosis complex, morphology of the smooth muscle cells, presence of PAS, PAS-D positive granules and HMB-45 immunoreactivity in tumor cells. Extramedullary hematopoiesis and hyaline globules are unique to hepatic AMLs. These specific pathologic manifestations not only implicate the histogenesis of AML from different organs, but also assist in making a correct diagnosis.

Selective sentinel lymph node dissection in breast cancer: experiences from Taiwan.

The significance of the sentinel lymph node (SLN) was examined in 58 Chinese breast cancer patients. The method of technetium-99m sulfur colloid injection and the intraoperative gamma probe was found to be very useful for identifying the SLN. The positive predictive value was 64.5%, and the negative predictive value was 93.2%. Findings suggest that lymph node dissection is not necessary in breast cancer patients with a negative SLN.

Benign metastasizing leiomyoma of the lung: a case report.

Benign metastasizing leiomyoma (BML) refers to benign pulmonary neoplasm associated with a previous or coincident history of uterine leiomyomata. We report the case of a 56-year-old postmenopausal woman with a 4-year history of multiple benign leiomyomatous lesions in bilateral lungs and the uterus. The tumor cells from the lungs and uterus were focally immunoreactive for HMB-45 antibody and progesterone receptor. Immunoreactivity to HMB-45 is well known in smooth muscle cells of hamartomatous neoplasms, such as angiomyolipoma and lymphangioleiomyomatosis, but has not been reported in BML previously. These features suggest a multifocal hamartomatous histogenesis rather than metastasis in the present case.