Blastic plasmacytoid dendritic cell neoplasm: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm.</p><p><b>METHODS</b>The clinical, morphology and immunophenotypic features were analyzed in 3 cases of blastic plasmacytoid dendritic cell neoplasm, with review of literature.</p><p><b>RESULTS</b>The pathologic changes of these tumors accorded with that of blastic plasmacytoid dendritic cell neoplasm, and they also had new characteristics, including lineage other than T, B, myeloid and NK cells, and immunophenotypes of CD56(+) CD4(-) CD123(+) TdT(+) CD43(+) CD68(+) , CD56(+) CD4(+) CD123(-) TdT(+) CD43(+) CD68(-) and CD56(+) CD4(+) CD123(-/+) TdT(-) CD43(+) CD68(+) in the 3 cases, respectively. Bone marrow involvement was found 5 years later in case 1, and was then stable after chemotherapy; case 2 and case 3 were died 5 and 2 months after diagnosis, respectively.</p><p><b>CONCLUSION</b>Blastic plasmacytoid dendritic cell neoplasm is a heterogeneous group of lymphoproliferative disorders, with different clinical, morphologic and immunophenotypic features.</p>

Rhabdomyosarcoma of the breast: a clinicopathologic study and review of the literature / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Rhabdomyosarcoma (RMS) is an uncommon malignancy of the breast. The aim of this study was to summarize its clinicopathologic features and biological behavior.</p><p><b>METHODS</b>Five primary or secondary breast RMSs were collected. Their clinicopathological characteristics and all published literature about breast RMS were reviewed. Immunohistochemical study of desmin, myogenic differentiation 1 (MyoD1), myogenin, leukocyte common antigen (LCA), vimentin, cytokeratin (AE1/AE3), E-cadherin, neuron specific enolase (NSE), CD99, chorioallantoic membrane 5.2 (CAM5.2) and epithelial membrane antigen (EMA) expression were performed.</p><p><b>RESULTS</b>The five patients were all female with ages ranging from 16 to 46 years old (mean, 30 years). Three were metastatic breast RMSs, two embryonal and one solid variant alveolar, with the primary tumor sites the right labium majus, left nasal meatus and nasopharynx, respectively. The other two, one embryonal and one alveolar, were primaries. Grossly, the surgical specimens revealed round or oval, well-demarcated but nonencapsulated masses. Their cut surfaces consisted of homogeneous grayish yellow or white tissue. Microscopically, most tumor cells were poorly differentiated small round, oval or small polygons with eosinophilic cytoplasm. All cases were positive for vimentin, desmin, MyoD1 and myogenin. One embryonal RMS also had a few cells with perinuclear staining of AE1/AE3. The other markers were negative.</p><p><b>CONCLUSIONS</b>Although primary or metastatic RMS in breast was almost confined to young adolescent females, our cases suggested that it can also happen to the middle-aged women. Embryonal RMS has a certain metastatic potential. MyoD1 and myogenin are two useful markers when making differential diagnosis. Axillary lymph node status and age may play a role in the prognosis of primary breast RMS patients.</p>

Invasive carcinoma arising in breast microglandular adenosis: a clinicopathologic study of three cases and review of the literature / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunophenotypes and differential diagnoses of invasive carcinoma arising in breast microglandular adenosis (MGACA).</p><p><b>METHODS</b>Clinical and pathologic findings of 3 cases of MGACA were analyzed by histomorphology and immunohistochemical staining of CK7, S-100 protein, ER, PR, HER2, SMA, MSA, p63 and PAS. Literatures were reviewed.</p><p><b>RESULTS</b>(1) Histologically, 3 tumors all showed a spectrum of glandular proliferations ranging from microglandular adenosis (MGA) to atypical microglandular adenosis (AMGA) to in situ carcinoma (DCIS) to invasive carcinoma. The invasive carcinoma component was ductal in case 1, and matrix-producing in case 2 and case 3. (2) All epithelial cells in MGA, AMGA, DCIS and MGACA were positive for CK7 and S-100 protein, but were negative for ER and HER2. PR was negative in case 1 and case 2 but was low positive in case 3. Myoepithelial cell differentiation was not demonstrated in MGA, AMGA, DCIS and MGACA by immunohistochemical staining for SMA, MSA or p63. PAS staining showed the presence of basement membrane in MGA, AMGA and DCIS, except MGACA.</p><p><b>CONCLUSIONS</b>MGACA is an extremely rare tumor of the breast and has distinct morphological and immunohistochemical features. Further studies are needed to evaluate the clinical behavior of this rare neoplasm.</p>

Epithelioid sarcoma-like hemangioendothelioma: a clinicopathologic and immunohistochemical study of 3 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of epithelioid sarcoma-like hemangioendothelioma (ES-H).</p><p><b>METHODS</b>The clinical, radiologic and pathologic features of three cases of ES-H were analyzed.</p><p><b>RESULTS</b>All the 3 cases occurred in male adults. The age ranged from 44 to 53 years. The presentations included left neck mass, iliac pain and bilateral shoulder masses. Histologically, ES-H was composed of a mixture of spindle and epithelioid tumor cells. Transition between the two cell types was demonstrated. The tumor cells were arranged in compact sheets, vague nodules or intersecting fascicles, amongst a collagenous stroma. Central coagulative necrosis was identified in one case, reminiscent the morphology that seen in epithelioid sarcoma. There was no evidence of angiogenesis, though focal presence of intracytoplasmic vacuoles was seen in one case, as in classic examples of epithelioid hemangioendothelioma. Immunohistochemical study showed that the tumor cells expressed both epithelial (AE1/AE3, CAM5.2 and epithelial membrane antigen) and endothelial (CD31, Fli-1 and factor VIII-related antigen) markers. Two of the cases were also positive for CD34. All of the patients were treated by surgical resection. Two patients remain well at 14-month and 9-month follow up, respectively. The remaining patient had repeated local recurrences during a 6-year period.</p><p><b>CONCLUSIONS</b>ES-H represents a rare morphologic type of hemangioendothelioma. It has some overlapping histologic features with epithelioid sarcoma and epithelioid hemangioendothelioma. The endothelial nature of ES-H is difficult to be verified on the basis of morphologic examination alone. Confirmation of the diagnosis with immunohistochemistry is necessary. ES-H is likely related to epithelioid hemangioendothelioma and may represent a cellular spindle cell variant of epithelioid hemangioendothelioma.</p>

Detection of chromosomal aberration in paraffin-embedded synovial sarcoma tissues by fluorescence in-situ hybridization / 中华病理学杂志

<p><b>OBJECTIVE</b>To evaluate the application of fluorescence in-situ hybridization (FISH) in detection of gene translocation in paraffin-embedded tissue samples of synovial sarcoma.</p><p><b>METHODS</b>Interphase FISH was carried out in paraffin-embedded tissue of 42 cases of synovial sarcoma and 9 cases of non-synovial sarcoma, using a LSI SYT (18q11.2) dual color break-apart probe. In all of the cases studied, the gene fusion product SYT-SSX was also analyzed by reverse transcription-polymerase chain reaction (RT-PCR).</p><p><b>RESULTS</b>Positive signals were detected in 37 cases (88.1%) of synovial sarcoma by FISH, as compared with 35 cases (83.8%) by RT-PCR and 39 cases (92.9%) by both techniques. Of the 39 positive cases, 33 cases (78.5%) revealed SYT gene translocation.</p><p><b>CONCLUSIONS</b>FISH may serve as an adjunctive diagnostic tool in problematic cases of synovial sarcoma and can be applied in paraffin-embedded tissue samples. As compared with RT-PCR, FISH is also sensitive and reliable. The methodology is less labor intensive and time consuming. FISH has great potential in molecular diagnosis of soft tissue tumors.</p>