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.

Focal myositis of the tongue presenting as macroglossia.

Focal myositis (FM) is a benign localized inflammatory process of unknown aetiology, which presents as a rapidly enlarging soft-tissue mass that evolves into a localized lesion. It is most often mistaken for a neoplasm. Histologically, it is characterized by marked myopathic changes of the muscle fibres, lymphocytic infiltration and interstitial fibrosis. We describe a case of focal myositis of the tongue and review this rare condition.

NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status.

AIMS: To analyse the clinicopathological findings of extranasal CD56+ cytotoxic T- or NK-cell lymphomas in different organs and to compare Epstein-Barr virus (EBV)+ and EBV- lymphoma of non-blastoid cytomorphology. METHODS AND RESULTS: Fifty-one cases of cCD3+ T-cell intracellular antigen (TIA-1)+ CD56+ lymphomas of extranodal/extranasal origin were included in the study. The primary sites of the CD56+ tumours were soft tissue (n = 10), the gastrointestinal (GI) tract (n = 13), the skin (n = 15), upper aerodigestive tract excluding nasal and nasopharyngeal regions (n = 11), the testis (n = 1), and parotid gland (n = 1). TCR gene rearrangement was detected in seven of 47 cases examined (16%). EBV was positive in 39 of 51 cases (76%). The positive rate of EBV was higher in tumours of soft tissue (80%), GI tract (92%), and skin (80%), and lowest in the upper aerodigestive tract excluding the nasal and nasopharyngeal region (50%). Tumours of the soft tissue and the upper aerodigestive tract tended to present with localized disease (P = 0.002). The 2-year survival rate was lowest for tumours of the GI tract (P = 0.0256). EBV- TCR- lymphoma showed less necrosis (P = 0.0133) and a better 2-year survival rate (P = 0.0066) than EBV+ TCR- lymphoma. Patients with EBV+ TCR+ lymphomas tended to present with localized disease, more often than EBV+ TCR- lymphoma (P = 0.0186). Significant prognostic factors in all CD56+ lymphomas were the site (P = 0.0256), EBV status (P = 0.0026), necrosis with or without perforation (P = 0.0338) and the presence of pleomorphic large tumour cells (P = 0.0428). Cox's regression analysis adjusting for other pathological parameters showed EBV status to be the only independent prognostic factor (P = 0.018). CONCLUSIONS: Extranodal CD56+ EBV- lymphoma at extranasal sites is a clinically less aggressive malignancy and displays less necrosis than CD56+ EBV+ lymphoma. Because CD56+ EBV+ TCR+ lymphomas show similar pathological and clinical findings to CD56+ EBV+ TCR- lymphomas, nasal-type NK/T-cell lymphomas at extranasal sites should be diagnosed as such on the basis of EBV+, cytotoxic T or NK phenotype irrespective of the genotype determined by molecular study.

Epstein-Barr virus-associated recurrent necrotic papulovesicles with repeated bacterial infections ending in sepsis and death: consideration of the relationship between Epstein-Barr virus infection and immune defect.

The disease of Epstein-Barr virus (EBV) -associated recurrent necrotic papulovesicles is a distinct clinicopathologic entity different from classic hydroa vacciniforme (HV). A few patients have been reported as atypical HV with systemic involvement, development of lymphoma, and poor prognosis. We describe a patient with recurrent necrotic papulovesicles and multiple varioliform scars in both sun-exposed and covered areas. In contrast to cases of previously reported atypical HV, our patient suffered from repeated bacterial infections on various sites ending in sepsis and death, but without malignant transformation. EBV was detected in the lymphoid cells from the skin lesions by anti-latent membrane protein (LMP) antibody and in situ hybridization. We suggest that the repeated bacterial infections in this case raise the possibility of an association of EBV infection with increased susceptibility to bacterial infections.

REAL classification of malignant lymphomas in the Republic of Korea: incidence of recently recognized entities and changes in clinicopathologic features. Hematolymphoreticular Study Group of the Korean Society of Pathologists. Revised European-American lymphoma.

BACKGROUND: The clinicopathologic characteristics of malignant lymphomas vary according to geography. The aim of this study was to determine the clinical characteristics of malignant lymphomas and the relative frequency in the Republic of Korea of lymphomas belonging to the newly described REAL (revised European-American lymphoma) classification categories. METHODS: The Hematolymphoreticular Study Group of the Korean Society of Pathologists conducted a consensus-based morphologic review of a nationwide collection of 1548 malignant lymphomas, including Hodgkin's disease (HD), diagnosed at 23 institutes over a recent 2-year period. Slides stained with hematoxylin and eosin or other immunohistochemical stains were reviewed. All cases were classified according to the histologic criteria proposed by the International Lymphoma Study Group. Clinical data, including age, gender, and site of disease involvement, were reviewed. RESULTS: The Republic of Korea had lower rates of HD and follicle center lymphoma and higher rates of extranodal lymphoma, diffuse large B-cell lymphoma, and angiocentric lymphoma compared with Western countries. The most frequent subtypes of non-Hodgkin's lymphoma (NHL) were diffuse large B-cell lymphoma, extranodal marginal zone B-cell lymphoma, peripheral T-cell lymphoma, unspecified type, and angiocentric lymphoma, in decreasing order. Compared with a previous nationwide study, the rates for follicular lymphoma and NHL involving the stomach, orbit, and central nervous system were increased. The rate of T-cell NHL in the current study was much lower than that reported in a previous study for the Republic of Korea and was also lower than that reported for other Far East countries. For HD, the relative frequency of nodular sclerosis subtype was higher compared with that in the previous study, and lymphocyte predominance and lymphocyte depletion were lower. CONCLUSIONS: The occurrence rates for various subtypes of malignant lymphoma in the Republic of Korea are distinct from those in Western countries and similar in many ways to those in other countries in the Far East. Compared with other Far East countries, the Republic of Korea has a higher rate of extranodal lymphoma, rare adult T-cell lymphoma, and a relatively low rate of T-cell lymphomas. It appears that over time there have been changes in the relative rates for follicular lymphoma, subtypes of Hodgkin's disease, and gastric and orbital lymphoma. The authors attribute these changes primarily to refined criteria for diagnosing new clinicopathologic entities.

Balo's concentric sclerosis: a clinical case study of brain MRI, biopsy, and proton magnetic resonance spectroscopic findings.

The antemortem diagnosis of Balo's concentric sclerosis was made in a 52 year old woman with subacute right hemiparesis on the basis of brain MRI and stereotactic brain biopsy, which showed multiple ring-like lesions of lamellated demyelination alternating with spared white matter. Proton magnetic resonance spectroscopy (1H-MRS) was carried out one and nine months after the onset of illness. The first 1H-MRS showed a decreased N-acetyl aspartate peak, an increased choline peak, presence of large lipid peaks, and high resonance at 1.4 ppm. The second 1H-MRS disclosed changes such as a decrease of lipid signal, a decrease of resonance at 1.4 ppm, and an increase in the myoinositol peak. These findings are similar to those reported for multiple sclerosis. It seems that this is the first report of 1H-MRS findings in Balo's concentric sclerosis.

Macrocystic serous cystadenoma of pancreas--a case report.

The macrocystic variant of serous cystadenomas of the pancreas has only recently been described. We present a case of a 40 year-old female, who presented with vague indigestion. The cyst was unilocular, and was lined by simple cuboidal, ciliated serous type epithelium. Fine needle aspiration, immunohistochemical, light microscopic, and electron microscopic studies are discussed.