T.W. Shek

Follicular dendritic cell sarcoma and interdigitating reticulum cell sarcoma: A review

Neoplasms of reticular dendritic origin are extremely rare and include the follicular dendritic cell sarcoma (FDCS) and the interdigitating reticulum (or dendritic) cell sarcoma (IDCS). In this article, we review the literature pertaining to the two diseases and describe clinical observations and salient pathologic features, including information provided by authors of FDCS and IDDCS reports. We performed a computerized database search for published articles regarding FDCS and IDDCS. The articles were evaluated critically by the authors. Simple descriptive statistics were used to analyze the data. There are 51 cases of FDCS and 21 cases of IDDCS that are well documented in the literature. The pathologic diagnosis of FDCS and IDDCS is often challenging and requires morphologic, immunophenotypic, cytochemical, and electron‐microscopic analysis. Patients with FDCS usually present with cervical or axillary lymphadenopathy, but extranodal disease has been described. In at least some patients, preexisting Castlemans disease has been recognized. Resected localized disease may be prevented from recurrence by consolidative radiotherapy. Chemotherapy regimens have shown nondurable antitumor activity in FDCS. Patients with IDDCS usually present with lymphadenopathy. The clinical course of IDDCS has been variable, but it seems to be more aggressive than that of FDCS. Variable degrees of remission may be achieved with chemotherapy. FDCS and IDDCS are rare neoplasms that may pose difficulty in pathologic diagnosis. IDDCS seems to display a more aggressive behavior than FDCS. Patients with IDDCS and FDCS can eventually die of disease progression. The role of chemotherapy and radiotherapy is not clearly defined. Am. J. Hematol. 59:161–167, 1998.

Diagnostic Significance of Mitochondria in Four Types of Renal Epithelial Neoplasms: An Ultrastructural Study of 60 Tumors

Mitochondrial morphology was studied in 60 renal epithelial neoplasms, including clear cell, papillary, chromophobe cell, and oncocytomas, to see if there are significant differences among the various subtypes. Mitochondria from the nephron tubular system obtained from 36 patients with glomerular diseases served as controls. Significant differences were found in mitochondrial ultrastructure in each of the subtypes of renal epithelial tumors. Novel observations were the presence of tubulovesicular cristae in many of the mitochondria found in chromophobe cell renal carcinomas and the discovery of a possible new oncocytic variant of this entity with mitochondria with pseudotubulovesicular and lamellar cristae similar to those of renal oncocytomas. This latter finding may be of prognostic significance, since renal oncocytomas are benign, while chromophobe cell neoplasms may behave in a malignant fashion.

Sustained disease remission after spontaneous HBeAg seroconversion is associated with reduction in fibrosis progression in chronic hepatitis B Chinese patients

Recently, controversies have arisen about whether hepatitis B e antigen (HBeAg) seroconversion can result in regression of fibrosis, thus improving the clinical outcome of Chinese patients with chronic hepatitis B. In this study, we determined if spontaneous HBeAg seroconversion is associated with regression of fibrosis in Chinese chronic hepatitis B patients. We evaluated the histology of liver samples from 128 HBeAg‐positive treatment‐naive Chinese patients who had undergone 2 liver biopsies over the years. Regression of fibrosis was defined as a decrease in fibrosis stage of at least 1 point. Sustained disease remission was defined as HBeAg seroconversion and hepatitis B virus (HBV) DNA < 104 copies/ml at follow‐up liver biopsy. The mean duration (± standard error of the mean) between the initial and follow‐up liver biopsies was 43.9 ± 3.4 months. Regression of fibrosis was higher in patients with sustained disease remission (5 of 13 [38.5%] versus 22 of 115 [19.1%], P < 0.00005), patients who were younger (20‐29 years old) at initial liver biopsy (17of 54 [31.5%] versus 10 of 74 [13.5%], P = 0.0004), and patients with genotype B (17of 43 [39.5%] versus 10 of 85 [11.8%], P = 0.004). On multivariate analysis, sustained disease remission (relative risk [RR] 3.00, 95% confidence interval [95% CI] 1.29‐7.01, P = 0.01) and being 20‐29 years old at initial liver biopsy (RR 2.94, 95% CI 1.01‐8.62, P = 0.04) were independently associated with regression of fibrosis. The rate of fibrosis progression was lower in patients with sustained disease remission than in those who remained HBeAg positive (median 0 fibrosis units/year, range −2.00 to −0.70 fibrosis units/year, versus median 0.51 fibrosis units/year, range 0 to +2.03 fibrosis units/year, P = 0.02). Conclusion: Spontaneous sustained remission of disease is associated not only with little progression of fibrosis but also with regression of fibrosis. (HEPATOLOGY 2007.)

Thermocoagulation of fetal sacrococcygeal teratoma

Fetal sacrococcygeal teratoma can lead to a high output cardiac failure resulting in hydrops fetalis. One of the prenatal therapeutic options is to occlude the feeding vessels by radiofrequency ablation. We present a case of fetal sacrococcygeal teratoma diagnosed at 13u2009weeks of gestation. The tumour increased in size more than 100‐fold over 5u2009weeks causing polyhydramnios and cardiac and placental enlargement. Thermocoagulation was performed at 18u2009weeks gestation by passing an insulated electric wire through an 18‐gauge needle placed close to the feeding vessels of the tumour at its neck. Blood supply to the tumour was successfully reduced. However, fetal death was diagnosed 2u2009days after the procedure. We speculate that it may be safer to limit the extent of coagulation in one attempt but to repeat the procedure at a later stage when necessary. Copyright

Ewing sarcoma of the small intestine.

This report describes a rare case of Ewing sarcoma (ES) of the small intestine. The patient was a 9-year-old girl with progressive abdominal distension. Computed tomography showed a large mass in the small bowel. Histopathologic examination of the resected tumor showed ES with typical histologic, immunohistochemical, and ultrastructural features. The tumor recurred in the pelvic cavity 18 months after the original surgery. Molecular study of the recurrent tumor confirmed a diagnostic EWS-FLI1 gene fusion. This patient illustrates the unique occurrence of ES in the small intestine.

Significance of Size of Lymph Node Metastasis on Postsurgical Stimulated Thyroglobulin Levels After Prophylactic Unilateral Central Neck Dissection in Papillary Thyroid Carcinoma

BackgroundThe prognostic significance of size of central lymph node metastasis (CLNM) in papillary thyroid carcinoma (PTC) remains unknown. Because postsurgical detectable stimulated thyroglobulin (DsTg) after radioiodine ablation may imply persistent or recurrent disease, we evaluated the association between size of CLNM and rate of DsTg in patients with PTC who underwent unilateral prophylactic central neck dissection.MethodsTo be eligible for analysis, the prophylactic central neck dissection specimen with <3 central lymph nodes (CLNs) or size of CLNM ≥1xa0cm as measured under the microscope was excluded. Of 132 specimens, 89 (67.4xa0%) were eligible. Forty patients (44.9xa0%) had no metastasis or pN0, 20 (22.5xa0%) had micrometastasis (<2xa0mm) or pN1mic and 29 (32.6xa0%) had macrometastasis (≥2xa0mm) or pN1mac. Postablation sTg level was measured 9xa0months after surgery. A multivariable analysis was conducted to identify independent factors for postablation DsTg.ResultsLarger-sized CLNM correlated significantly with younger age (pxa0=xa00.028), greater number of CLN retrieved (pxa0=xa00.016), greater number of metastatic CLN excised (pxa0<xa00.001), higher metastatic CLN ratio (pxa0=xa00.006) and postablation sTg level (pxa0=xa00.012). In the multivariable analysis, after adjusting for tumor size and metastatic CLN ratio, size of CLNM was an independent predictor of postablation DsTg (odds ratio 1.56, 95xa0% confidence interval 1.09–2.24, pxa0=xa00.015). Relative to pN0, the odds ratios for postablation DsTg in pN1mic and pN1mac were 2.53 (95xa0% confidence interval 0.35–19.00, pxa0=xa00.351) and 5.81 (95xa0% confidence interval 1.22–27.70, pxa0=xa00.027), respectively.ConclusionsSize of CLNM was an independent factor for DsTg 9xa0months after surgery. Patients with pN1mac were almost 6 times more likely to have postablation DsTg than those with pN0 or pN1mic.

Composite paraganglioma-ganglioneuroma of the urinary bladder: A clinicopathologic, immunohistochemical, and ultrastructural study of a case and review of the literature

Urinary bladder paragangliomas are rare. An 81-yr-old woman was admitted because of whole-stream painless hematuria. Investigations revealed a pedunculated bladder tumor. Pathological examination showed a biphasic tumor, composite paraganglioma-ganglioneuroma. The two separate components were documented by both immunohistochemical and ultrastructural studies. A review of the English literature on urinary bladder paragangliomas showed that the present case is the first case with this unique feature documented in detail, and the patient is the oldest one being reported.

Cumulative hepatotoxicity induced by continuous low- dose cyclophosphamide therapy

TO THE EDITOR: Cyclophosphamide (CYC) is an alkylating agent used in the treatment of a number of rheumatological and malignant diseases. Well-documented toxicities include alopecia, mucositis, nausea, vomiting, marrow suppression, cystitis, and gonadal failure. Although hepatotoxicity is not uncommon during high-dose CYC therapy, hepatitis induced by low-dose treatment has rarely been described (1, 2, 3, 4, 5, 6, 7, 8). In the latter cases, hepatotoxicity occurs exclusively within the first 2–8 wk of therapy, at a dosage of 50–180 mg/day. We report a patient with primary Sjogrens syndrome who developed acute hepatitis 2 yr after a total cumulative dose of 40.5 g of CYC. To our knowledge, this cumulative toxic effect of CYC on the liver during continuous low-dose administration has hitherto been undescribed.

Factors affecting the adequacy of lymph node yield in prophylactic unilateral central neck dissection for papillary thyroid carcinoma.

Background n nDeterminants for adequate lymph node yield (LNY) in prophylactic central neck dissection (pCND) for papillary thyroid carcinoma (PTC) remain unclear. We aimed to determine factors affecting LNY in pCND. n n n nMethods n nOf 230 patients, 109 (47.4%) had total thyroidectomy and unilateral pCND. A specimen of ≥6 central lymph nodes (CLNs) was considered adequate. Factors such as patient clinicopathologic features, specimen dimensions, and pathologists experience were compared between those with LNYu2009<u20096 (nu2009=u200952) and LNYu2009≥u20096 (nu2009=u200957). A multivariate analysis was conducted to identify independent factors for LNYu2009≥u20096. n n n nResults n nAge, sex, presentation, body mass index, tumor characteristics, TNM stages, MACIS score, and pathologists experience were not significant determinants for LNYu2009≥u20096. In the univariate analysis, the length (Pu2009=u20090.021), width (Pu2009=u20090.047), thickness (Pu2009=u20090.024), and pN1a (Pu2009=u20090.042) were significant determinants but in the multivariate analysis, the length (ORu2009=u20091.486 (95% CI: 1.053–2.097), Pu2009=u20090.024) was the only independent factor for LNYu2009≥u20096. Postoperative vocal cord palsy, hypoparathyroidism, stimulated thyroglobulin and recurrences were similar between LNY <6 and ≥6. n n n nConclusions n nLength (or the longest measured dimension) of the fresh CLN specimen was the only factor assuring LNYu2009≥u20096. Surgical complications and short-term outcomes appeared similar between LNY <6 or ≥6. J. Surg. Oncol. 2012; 106: 966–971.

Post-transplantation lymphoproliferative disease in Chinese: The Queen Mary Hospital experience in Hong Kong

Post-transplantation lymphoproliferative disease (PTLD) is an unique iatrogenic complication after bone marrow transplantation (BMT) and solid organ transplantation (SOTx). The pattern of EBV related lymphoma in Chinese is different from Caucasians. We surveyed the incidence, clinical and pathological spectrum of PTLD among 541 cases of allogeneic BMT, 145 cases of renal transplant, 35 cases of heart/lung transplantation and 146 cases of orthotopic liver transplantation (OLT). From 1994 to 2001, 13 consecutive cases of PTLD were diagnosed, ranging from disseminated NK cell lymphoma to localized plasmacytoma. Both donor and recipient derived PTLD was documented. Disease was often heralded by cytomegaloviral disease and antithymocyte globulin (ATG) usage. Two cases were diagnosed post-mortem, and six patients died of PTLD at a median of 3 months. Complete and partial remission was only achieved in 3 and 2 cases, respectively, despite a range of treatment (reduced immuosuppression, explantation, radiotherapy, combination chemotherapy, donor lymphocytes, autologous marrow infusion and rituximab). Most responding patients died subsequently of rejection, infection and graft versus host disease (GVHD). The incidence of PTLD is not increased in Chinese patients. However, some patients may be at increased risk, especially mismatched allogeneic BMT, parental OLT (especially involving young infants) and heavy ATG exposure.