Successful Management of Primary Mediastinal Large B-cell Lymphoma during Pregnancy.

We experienced a pregnant woman with superior vena cava syndrome at 15 weeks of pregnancy who was diagnosed with primary mediastinal large B-cell lymphoma and given chemotherapy. In this case, the clinical courses of both the mother and infant were favorable without any serious complications because of close multidisciplinary cooperation. Based on a retrospective review of this case, the administration of CHOP-like regimens during the second and third trimesters appears relatively safe. Because pregnancy and continuation of pregnancy are rare in patients with hematopoietic malignancies, the accumulation of detailed information is important.

Possible Relationship Between MYBL1 Alterations and Specific Primary Sites in Adenoid Cystic Carcinoma: A Clinicopathological and Molecular Study of 36 Cases.

BACKGROUND: Adenoid cystic carcinoma (ACC) is a relatively rare malignant neoplasm that occurs in salivary glands and various other organs. Recent studies have revealed that a significant proportion of ACCs harbor gene alterations involving MYB or MYBL1 (mostly fusions with NFIB) in a mutually-exclusive manner. However, its clinical significance remains to be well-established. METHODS: We investigated clinicopathological and molecular features of 36 ACCs with special emphasis on the significance of MYBL1 alterations. Reverse-transcription polymerase-chain reaction (RT-PCR) and fluorescence in-situ hybridization (FISH) were performed to detect MYB/MYBL1-NFIB fusions and MYBL1 alterations, respectively. Immunohistochemistry was performed to evaluate MYB expression in the tumors. The results were correlated with clinicopathological profiles of the patients. RESULTS: RT-PCR revealed MYB-NFIB and MYBL1-NFIB fusions in 10 (27.8%) and 7 (19.4%) ACCs, respectively, in a mutually-exclusive manner. FISH for MYBL1 rearrangements was successfully performed in 11 cases, and the results were concordant with those of RT-PCR. Immunohistochemically, strong MYB expression was observed in 23 (63.9%) tumors, none of which showed MYBL1 alterations. Clinicopathologically, a trend of a better disease-specific survival was noted in patients with MYBL1 alterations than in those with MYB-NFIB fusions and/or strong MYB expression; however, the difference was not significant. Interestingly, we found tumors with MYBL1 alterations significantly frequently occurred in the mandibular regions (P = 0.012). Moreover, literature review revealed a similar tendency in a previous study. CONCLUSION: Our results suggest that there are some biological or etiological differences between ACCs with MYB and MYBL1 alterations. Moreover, the frequent occurrence of MYBL1-associated ACC in the mandibular regions suggests that MYB immunohistochemistry is less useful in diagnosing ACCs arising in these regions. Further studies are warranted to verify our findings.

Vanishing Parotid Tumors on MR Imaging.

BACKGROUND: Of all parotid gland tumors, only oncocytoma has been reported to appear isointense to the parotid gland, namely vanishing, on fat-saturated T2 and T1 postcontrast gadolinium-enhanced magnetic resonance imaging (MRI). The purpose of this study was to evaluate vanishing of parotid tumors on conventional MRI with and/or without postcontrast gadolinium-enhancement and on diffusion weighted imaging (DWI). METHODS: In 8 of 51 patients, ten parotid gland tumors had homogeneously enhanced lesions and were retrospectively analysed. Comparisons of signal intensity between those parotid tumors and parotid glands and evaluations of vanishing were performed on T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), fat-suppressed T2WI (FS-T2WI), postcontrast gadolinium-enhanced T1WI (CE-T1WI) and fat-suppressed CE-T1WI (FS-CE-T1WI), DWI as well as apparent diffusion coefficient (ADC). RESULTS: Ten parotid gland tumors consisted of five Warthin tumors, two pleomorphic adenomas, two parotid carcinomas (small cell carcinoma and adenoid cystic carcinoma) and one oncocytoma. All tumors showed hypointensity on T1WI and hyperintensity on DWI. Nine of ten tumors showed vanishing on the other MR sequences. All Warthin tumors showed vanishing on FS-T2WI, FS-CE-T1WI and the ADC map. One oncocytoma showed vanishing on FS-T2WI and the ADC map and hyperintensity on FS-CE-T1WI. All pleomorphic adenomas showed vanishing on T2WI and CE-T1WI. One adenoid cystic carcinoma showed vanishing only on CE-T1WI. CONCLUSION: Vanishing of parotid tumors can be observed not only on FS-T2WI and FS-CE-T1WI but also on T2WI, CE-T1WI and ADC mapping.

Differentiating parotid tumors by quantitative signal intensity evaluation on MR imaging.

BACKGROUND: There have been no reports about quantitative evaluations of enhancing components of parotid tumors on conventional MR imaging. PURPOSE: To evaluate the signal intensity of the enhancing components of tumors, including pleomorphic adenomas (PAs), Warthin tumors (WTs) and malignant tumors (MTs), on various MR sequences and to assess the usefulness of quantitative evaluations for differentiation among the three groups of tumors. MATERIAL AND METHODS: A total of 39 histologically proven tumors, including 15 PAs, 17 WTs and 7 MTs, were enrolled in this study. The tumor-to-spinal cord contrast ratio (TSc-CR) was calculated by dividing the signal intensity of the lesion by that of the spinal cord on MR sequences, including T1-weighted imaging (T1WI), T2-weighted imaging (T2WI) and postcontrast gadolinium-enhanced T1WI (CE-T1WI). The mean apparent diffusion coefficient (ADC) value was selected in each tumor. Furthermore, the differences in the TSc-CRs and the ADC values among the three groups of tumors were statistically evaluated. Cutoff values were determined for the prediction of tumor histology. RESULTS: The TSc-CRs of PAs were significantly higher than those of WTs and MTs on T2WI and CE-T1WI. The sensitivities and specificities were 100% and 87.5%, respectively, at a cutoff value of 1.31 for the TSc-CR of T2WI; and 83.3% and 100%, respectively, at a cutoff value of 2.00 for the TSc-CR of CE-T1WI. For the ADC values, sensitivity and specificity for discriminating PAs from WTs or MTs were both 100% when the cutoff value of the ADC was set at 1.40×10-3mm2/s. CONCLUSION: ADC maps and the quantitative evaluations using the TSc-CRs on T2WI and CE-T1WI were useful for discriminating WTs or MTs from PAs. However, discrimination between WTs and MTs was difficult using any MR sequence.

Acute undifferentiated leukemia (stem cell acute leukemia) showing differentiation to Langerhans cell-like cells in lymph nodes.

A 75-year-old woman was referred to our hospital with suspected leukemia. Complete blood count demonstrated WBC 3,810/µl with 26% blasts, Hb of 11.7 g/dl and Plt of 18.0×104/µl. Bone marrow aspiration revealed blasts (86.3%) with expressions of CD34, CD7, TdT, CD33, and CD117. MPO was negative. Chromosomal analysis of the bone marrow showed isolated trisomy 10 in all leukemic cells (20/20). Swelling of superficial lymph nodes was also observed. Cervical lymph node biopsy revealed leukemic blasts which had the same phenotype as those in the bone marrow except that proliferation of Langerhans cell-like cells (LCs) was observed in the paracortex. LCs had pale cytoplasm and grooved nuclei, and were positive for both CD1a and S100 protein. Trisomy 10 was detected in both the leukemic blasts and the LCs by fluorescence in situ hybridization. This rare case strongly suggests leukemic cells to differentiate into LCs.

Isolated port-site metastasis of hepatocellular carcinoma after laparoscopic liver resection.

Port-site metastasis of hepatocellular carcinoma (HCC) is extremely rare, and only one case has been reported in the English-language literature. Contamination with malignant cells along the needle tract during percutaneous biopsy or radiofrequency ablation is a well-recognized cause of HCC recurrence. Here, we describe a case of port-site metastasis after laparoscopic liver resection of HCC. The patient, who had undergone laparoscopic partial resection of the left lateral segment of the liver 18 months earlier, was diagnosed with HCC. CT showed a nodule in the abdominal wall where the laparoscopic port had been inserted during resection. Local excision was performed, and histological examination revealed HCC consistent with recurrence after laparoscopic resection. The experience described in this report highlights the risk of port-site metastasis of HCC. Imaging for oncologic surveillance after laparoscopic resection must include all port sites.

High viral load of Merkel cell polyomavirus DNA sequences in Langerhans cell sarcoma tissues.

BACKGROUND: Langerhans cell (LC) sarcoma (LCS) is a high-grade neoplasm with overtly malignant cytologic features and an LC phenotype. We very recently suggested that LC behaves as a reservoir for common dermotropic Merkel cell polyomavirus (MCPyV) and determined the relationship between LC histiocytosis (LCH), which has an underlining oncogenic capacity, and MCPyV as a trigger for a reactive process rather than a neoplastic process. We propose LC to be a reservoir for MCPyV and hypothesize that some LCS subtypes may be related to the MCPyV agent. FINDINGS: We examined seven LCS tissues using multiplex quantitative PCR (Q-PCR) and immunohistochemistry with anti MCPyV large-T (LT) antigen antibody. High viral loads of MCPyV DNA sequences (viral load = relative levels of MCPyV) were detected (0.328-0.772 copies/cell (Merkel cell carcinoma (MCC) = 1.0)) using Q-PCR in 43% (3/7) tissues, but LT antigen expression was not observed (0/7). CONCLUSIONS: Frequent MCPyV-DNA amplification suggests that LCS in some patients may be related to MCPyV infection. Moreover, the higher viral load of LCS (median, 0.453 copies/cell) than low load of LCH (0.003, median of 12 cases) (P < 0.01) may suggest a virally induced tumorigenic process in some LCS. Although the absence of LT antigen expression may indicate a different role for MCPyV in this pathology, some subtypes of LCS may develop in the background of MCPyV-infected LC. To the best of our knowledge, this is the first report on the relationship between MCPyV and LCS. The recent discovery of MCPyV opened new therapeutic avenues for MCC. These data open novel possibilities for therapeutic interventions against LCS.

CD5-negative mantle cell lymphoma resembling extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue : a case report.

A 71-year-old male underwent an upper gastrointestinal endoscopy ; as a result of a biopsy, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) was suspected. Abdominal computed tomography scan disclosed an approximately 4-cm-large mass in the ileocecal region. After ileocecal resection, the patient was diagnosed with MALT lymphoma (CD79a(+), CD20(+), CD3(-), CD5(-), CD10(-), and cyclin D1(-)). He achieved complete remission after receiving chemotherapy. However, four years after the primary onset, he was diagnosed with recurrence. Although he achieved remission again by salvage therapy, six years after the primary onset, he was referred to our hospital with second recurrence. Colonoscopy revealed the appearance of multiple lymphomatous polyposis and biopsy specimens showed monotonous proliferation of centrocyte-like cells (CD79a(+), CD20(+), CD3(-), CD5(-), CD10(-), and cyclin D1(+)), which were consistent with mantle cell lymphoma (MCL) except for CD5. The result of reactivity to cyclin D1 was different from that at initial diagnosis, so we reexamined the initial surgical specimens, the histological and histochemical features of which were proven to be the same as those of colonic biopsy specimens. Finally, the patient was diagnosed with CD5-negative MCL (marginal zone-like variant). As MALT lymphoma and MCL sometimes show similar histological features, they are difficult to distinguish from each other. It is necessary to take the possibility of this rare phenotype of MCL into consideration and to reexamine the initial diagnosis, especially if the clinical course is unusual for MALT lymphoma. This case is very interesting in view of its indolent clinical feature and phenotype.

Biphasic metaplastic sarcomatoid carcinoma of the breast: report of a case.

A 69-year-old woman was admitted to our hospital with the complaint of a right breast mass. As a result of thorough examinations, she was diagnosed with breast cancer and underwent breast-conserving surgery. The pathological findings of the resected specimen showed that the tumor consisted of intermingled carcinomatous and sarcomatous components with a transition zone. On immunohistochemical study, the sarcomatous cells in this transition zone showed partial positive staining for CD10, the myoepithelial marker, suggesting that the myoepithelial cells had transformed into sarcoma, and then this biphasic tumor was formed. Finally, she was diagnosed with biphasic metaplastic sarcomatoid carcinoma of the breast. Biphasic metaplastic sarcomatoid carcinoma of the breast is a relatively rare but aggressive disease. The pathological diagnosis is often controversial, requiring detailed immunohistochemical analysis. We report our experience with a case of biphasic metaplastic sarcomatoid carcinoma of the breast.

Transvaginal ultrasonography and endometrial cytology as a diagnostic schema for endometrial cancer.

OBJECTIVE: To determine whether the combined use of transvaginal ultrasonography and endometrial cytology is an effective diagnostic schema for endometrial cancer and hyperplasia of the uterus. METHODS: Five hundred fifty-two women were enrolled in this study. For all subjects, endometrial thickness was evaluated by transvaginal ultrasonography. Endometrial cytology was carried out according to the following criteria: all women with atypical uterine bleeding, for asymptomatic postmenopausal women with endometrial thickness >or=5 mm, and for asymptomatic premenopausal women with endometrial thickness >or=20 mm. When the cytological findings were abnormal, we performed a hysteroscopy-guided biopsy within 2 weeks. Women who received transvaginal ultrasonography alone, or those who showed negative cytology, underwent repeated gynecological examination and transvaginal ultrasonography 3, 6, and 12 months after the first examination. RESULTS: Endometrial cytology was done on 129 women (23.4% of all subjects), of whom 14 were diagnosed as 'positive' cytology, 20 as 'suspicious positive', and 95 as 'negative'. A total of 34 women with 'positive' or 'suspicious positive' cytological result underwent hysteroscopy-guided endometrial biopsy. The histological diagnosis of the endometrium included 13 endometrial cancers, 9 endometrial hyperplasias (one atypical hyperplasia and 3 hyperplastic polyps), and 10 normal endometria. As a diagnostic schema for endometrial cancer, this combined method resulted in 100% sensitivity, 99.1% specificity, 92.9% positive predictive value, and 100% negative predictive value. For endometrial hyperplasia, the method resulted in 100% sensitivity, 89.6% specificity, 40.0% positive predictive value, and 100% negative predictive value. CONCLUSION: A combination of transvaginal ultrasonography and endometrial cytology may be an effective diagnostic schema for endometrial cancer and hyperplasia.

Cardiac chondrosarcoma producing parathyroid hormone-related protein.

Chondrosarcoma is a malignant tumor characterized by the formation of cartilage. A case of primary cardiac chondrosarcoma of the left atrium developed in a middle-aged male. The preoperative serum concentrations of C-parathyroid hormone-related protein (PTHrP) and calcium were high (413.2 pmol/L and 12.2 mg/dl, respectively), but normalized after resection of the tumor, which measured 7 x 5 x 3.5 cm. The tumor was histopathologically diagnosed as chondrosarcoma, composed of outer atypical chondroid cells and inner pleomorphic and spindle mesenchymal cells mimicking malignant fibrous histiocytoma. Half of the cartilaginous tumor cells and a few pleomorphic cells showed cytoplasmic immunoreactivity for PTHrP. The tumor is a possible example of the functional pleiotropy of chondrosarcoma.

Acral pseudolymphomatous angiokeratoma of children: immunohistochemical and clonal analyses of the infiltrating cells.

BACKGROUND: Acral pseudolymphomatous angiokeratoma of children (APACHE) is a disorder characterized clinically by red nodules and histopathologically by a massive subepidermal lymphohistiocytic infiltrate. Although it was initially thought to be a vascular nevus, it has never been regarded as a pseudolymphoma. CASE REPORT: We report a 7-year-old-girl with small red nodules on the dorsum of the right foot and a 73-year-old man with asymptomatic brown-red nodules on the lower extremities. RESULTS: Histopathologic examination revealed a massive lymphohistiocytic infiltrate with plasma cells, some eosinophils, or a multinucleated giant cell immediately beneath the epidermis. Thick-walled vessels were observed in the infiltrate. These characteristics are identical to those of acral pseudolymphomatous angiokeratoma of children. The infiltrate was composed mainly of equal numbers of CD4+ or CD8+ T cells and equal numbers of B cells stained for kappa or lambda light chains. PCR amplification of rearranged immunoglobulin heavy chain genes or T-cell receptor gamma genes showed no evidence of clonality, suggesting that these infiltrates were polyclonal both for B and T cells. CONCLUSIONS: Our data support the idea that this disorder represents a reactive process. The modified term 'papular angiolymphoid hyperplasia' would define this disorder more appropriately.