Extranodal natural killer/T-cell lymphoma from skin or soft tissue: suggestion of treatment from multinational retrospective analysis.

BACKGROUND: Clinical features and outcomes of extranodal natural killer/T-cell lymphoma (ENKL) arising from extranasal sites are not fully understood. The purpose of this study was to study the prognosis and treatment outcome of skin/soft tissue primary ENKL. PATIENTS AND METHODS: This multicenter retrospective study included 48 patients with skin/soft tissue primary ENKL diagnosed from 1993 to 2010. RESULTS: Patients with Ann Arbor stage I, T1-2N0M0 by International Society for Cutaneous Lymphomas-European Organization of Research and Treatment of Cancer TNM (tumour-node-metastasis) stage, International prognostic index score of 0-1, and a Korean prognostic index (KPI) score of 0-1 were associated with better survival. Four of five patients with T1-2N0M0 disease achieved complete response with radiation alone. In disseminated disease, only 6 of 13 patients responded to anthracycline-containing chemotherapy, and all the two patients receiving SMILE showed response. CONCLUSION: In conclusion, we identified the prognostic value of KPI, and we suggest a treatment recommendation according to the TNM (tumour-node-metastasis) stage. Radiotherapy with/without chemotherapy seemed to be optimal in localized disease. In advanced stages, a more aggressive treatment regimen with newer agents should be sought.

Perforation predicts poor prognosis in patients with primary intestinal diffuse large B-cell lymphoma.

AIMS: To elucidate the clinicopathological features and prognostic factors of primary intestinal diffuse large B-cell lymphoma (PI-DLBL). METHODS AND RESULTS: Archival tissues from 30 tumours were used for tissue microarray construction, immunohistochemistry and interphase fluorescence in situ hybridization for chromosomal translocation. The M:F ratio was 1.7:1, with a median age of 60 years. The ileum and ileocaecum were most frequently involved (40% each). Fourteen (47%) were at stage I(E) disease, 15 (50%) at stage II(E). Five (17%) tumours were perforated at presentation. The tumours expressed Bcl-6 (73%), MUM1 (70%), Bcl-2 (67%) and CD10 (23%). Nine (30%) were classified as germinal centre B-cell (GCB) phenotype and 21 non-GCB. Eight of 30 (27%), 7/30 (23%) and 2/29 (7%) cases were positive for rearrangements involving IGH, BCL6, and C-MYC loci, respectively, whereas all cases were negative for BCL2 and CCND1 translocation. Perforation was a poor prognostic indicator, with a hazard ratio of tumour-related death at 8.75 (P = 0.001). The differentiation antigens, GCB versus non-GCB phenotype, or lymphoma-associated translocations were of no prognostic significance. CONCLUSIONS: We found a higher rate of perforation and lower frequency of GCB phenotype in PI-DLBL in Taiwan compared with other geographical areas; perforation is a poor prognostic indicator.

Sporadic paediatric and adult Burkitt lymphomas share similar phenotypic and genotypic features.

AIMS: To characterize the clinicopathological features of sporadic Burkitt lymphoma (BL). METHODS AND RESULTS: A retrospective study of 17 paediatric and 14 adult BLs with history and histopathology review, immunohistochemistry, Epstein-Barr virus (EBV) in situ hybridization (EBER) and fluorescence in situ hybridization. There was no statistically significant difference in gender, frequency of central nervous system (CNS) involvement and leukaemic change at presentation, or frequency of CD10+/Bcl-2-/Bcl-6+ (88% versus 86%), Ki67 labelling index, EBER (24% versus 21%), or C-MYC translocation (100% versus 92%) between paediatric and adult tumours. Correct pretreatment diagnoses were made in 13/17 (76%) paediatric and in 9/14 (64%) adult tumours. Twenty-eight patients received chemotherapy including 13/16 (81%) paediatric and 3/12 (25%) adult patients with appropriate regimens; 16 (57%) received CNS prophylaxis. The 1- and 5-year overall survival (OS) rates for paediatric patients were 80% and 50%, respectively, whereas 1-year OS for adults was 15%. CONCLUSIONS: Sporadic paediatric and adult BLs were phenotypically and genotypically similar. The significant prognosticators were age (P = 0.001), with or without CNS prophylaxis (P = 0.004), and CNS involvement (P = 0.008) and leukaemic change (P = 0.019) in disease course. The poor outcome in adult patients might be related to incorrect diagnosis and inappropriate treatment.

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.

Endoscope-assisted microsurgery for treatment of a suprasellar craniopharyngioma presenting precocious puberty.

Being the most common childhood tumor to involve the hypothalamus and pituitary gland, craniopharyngioma, a histologically benign tumor, is usually related to growth retardation and hypogonadism. The presentation of precocious puberty is very rare for a hypothalamic craniopharyngioma. Here, we report such a case. The female patient had presented with symptoms of menarche and breast development since she was 6 years old. Hormonal therapy with a gonadotropin-releasing hormone analogue was instituted to cease precocious puberty but was unsuccessful. Magnetic resonance imaging of the sella showed a 1-cm tumor in the hypothalamic area. Through a pterional approach, the tumor was removed en bloc with endoscope-assisted microsurgery. After the operation, our patient's symptoms improved, so the hormone therapy was discontinued. This extremely unique association of craniopharyngioma and precocious puberty, the causes and mechanisms involved along with the advantages of endoscope-assisted microsurgery are discussed.

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.