Ginandroblastoma multiloculado, negativo para CD99 en una niña de 12 años: caso clínico / A twelve-years-old girl with multiloculated gynandroblastoma CD99 negative. Case report

Los ginandroblastomas son tumores del ovario extremadamente raros, los cuales comparten componentes de células de la granulosa y de células de Sertoli/Leydig. Se describe un caso de una niña de 12 años, quien presenta hemorragia uterina anormal y sensación de masa intraabdominal de crecimiento progresivo asociado a menorragia, niveles de CA-125 en 60,4 UI/mL y estudios de extensión que reportan masa quística en ovario izquierdo, manejada con ooforectomía. El estudio anatomopatológico muestra un tumor multiloculado lleno de material seroso, abundantes cuerpos de Call-Exner y 45% de células de Sertoli/Leydig. La inmunohistoquímica reveló inmunorreactividad para inhibina, calretinina y pCK, mientras que los marcadores CD99 y AE1/AE3 fueron negativos. Se trata del primer reporte de caso sobre un ginandroblastoma multiloculado, negativo para CD99 en una niña de 12 años, estudio que plantea un abordaje sistemático para los tumores de las células de los cordones sexuales.

The ginandroblastoma is an extremely rare ovarian tumor which shows components of granulosa cells and Sertoli/Leydig cells. We describe a case of a twelve-years-old girl who presented abnormal uterine bleeding and progressively growing intra- abdominal mass associated with menorrhagia, CA-125 60.4 UI/mL and extension studies reporting cystic mass in the left ovary. She underwent oophorectomy. Pathological study shows a multilocular tumor filled with serous material. Many Call-Exner bodies were observed in the histopathological analysis, 45% of Sertoli/Leydig cells. Immunohistochemistry was reactive for inhibin, calretinin and pCK while AE1/AE3 and CD99 markers were negative. This is the first case report about a multiloculated gynandroblastoma, negative for CD99 in a 12-years-old girl. Thus, the study of this clinical case represents a systematic approach for tumors of the sex cord cells.

CD99 regulates redifferentiation of classical Hodgkin's lymphoma cell line L428 towards B cells / 南方医科大学学报

<p><b>OBJECTIVE</b>To explore the effect of CD99 overexpression on the morphology and differentiation-related phenotypes of classical Hodgkin's lymphoma cell line L428 and investigate the role of CD99 gene in Hodgkin/Reed-Sternberg (HRS) cell generation and transformation.</p><p><b>METHODS</b>The effect of CD99 overexpression on the cell morphology was detected by HE staining and phalloidin staining. Differentiation-related protein expressions were detected by immunocytochemistry and flow cytometry after stable transfection of CD99 gene in L428 cells.</p><p><b>RESULTS</b>CD99 overexpression caused a decrease of the cell size and reorganization of the actin cytoskeleton in L428 cells. Upregulation of CD99 led to the loss of classical Hodgkin's lymphoma diagnosis marker CD30 and CD15 and the restoration of the B-cell makers of PAX5, CD19, CD79α, BCL-6, and CD10.</p><p><b>CONCLUSION</b>CD99 overexpression leads to redifferentiation of L428 cells towards B cells, suggesting that the loss of B-cell phenotype in classical Hodgkin's lymphoma is very likely a result of down-regulated CD99 expression.</p>

Evaluation of the expression and significance of Claudin-5 and CD99 in solid-pseudopapillary neoplasms and neuroendocrine tumors of pancreas / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the expression of endothelium tight junction protein Claudin-5 and intercellular adhesion molecule CD99 in solid-pseudopapillary neoplasms (SPN) and neuroendocrine tumors of pancreas (P-NET), and their significance in the differential diagnoses.</p><p><b>METHODS</b>Immunohistochemical staining of Claudin-5 and CD99 was performed in 37 cases SPN and 21 cases of P-NET.</p><p><b>RESULTS</b>Membranous Claudin-5 expression was observed in all cases of SPN but was absent in all cases of P-NET. The difference was significant (P < 0.01). In SPN, 91.9% (34/37) of the cases displayed paranuclear dot-like immunoreactivity for CD99; in contrast, 61.9% (13/21) of the cases of P-NET displayed membranous staining (P < 0.01). There was a positive association between the expression of Claudin-5 and CD99 in SPN (r = 0.421,P = 0.001).</p><p><b>CONCLUSIONS</b>Although the macroscopic and microscopic features of SPN are quite characteristic, they may not allow confident differentiation from P-NET in all cases, especially when these characteristics are not classical. If necessary, immunostaining for Claudin-5 and CD99 can help to differentiate between these entities.</p>

A case of hemangiopericytoma of the soft palate with articulate disorder and dysphagia / 国际口腔科学杂志·英文版

We report a case of hemangiopericytoma of the soft palate of 60-year-old patient, who noticed a mass of the soft palate and experienced difficulty in speaking. We found a pediculate, hard, elastic mass measuring 38 mm (cross-sectional diameter). Computed tomography (CT) scans and dynamic magnetic resonance imaging (MRI) confirmed irregularly shaped mass and revealed a heterogeneous internal composition, consistent with vascular tumors. We excised the tumor under general anesthesia. Histopathological diagnosis was based on positive immunoreactivity of CD99 and vimentin and weak, positive staining of CD34. Three and half years following tumor excision, there is no recurrence or metastasis.

Small cell malignant tumors of bone: comparison between diagnosis using core needle biopsies and surgical specimens / 中华病理学杂志

<p><b>OBJECTIVE</b>To compare the pathologic diagnosis and immunohistochemistry of small cell malignant tumors (SCMT) of bone using both core needle biopsy and surgical specimen.</p><p><b>METHODS</b>Seventy-seven cases of SCMT with core needle biopsies and surgical specimens available were respectively analyzed by histologic examination and immunohistochemical study, with literature review.</p><p><b>RESULTS</b>The male-to-female ratio was 48:29. The age of the patients ranged from 6 to 73 years. The tumors studied included Ewing sarcoma/PNET (n = 38), myeloma (n = 23), lymphoma (n = 10), small cell osteosarcoma (n = 2), small cell carcinoma (n = 2) and mesenchymal chondrosarcoma (n = 2). The tumors involved limbs, axial skeleton and flat bones. Microscopically, the tumors shared similar histology, with small round cells and spindly cells arranged in diffuse sheets. The pathologic diagnosis by core needle biopsies correlated with that by surgical specimens in 84.4% (65/77) of the cases.</p><p><b>CONCLUSIONS</b>SCMT represents a heterogeneous group of malignancy. Correlations with clinicoradiologic findings and application of ancillary investigations including immunohistochemistry and molecular study are important for definitive diagnosis. Pathologic diagnosis using core needle biopsies shows good results and provides useful information for surgical planning.</p>

Primitive neuroectodermal tumor in female genital tract: a clinicopathologic study / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features of primitive neuroectodermal tumor (PNET) in female genital tract.</p><p><b>METHODS</b>Six cases of PNET arising in female genital tract were retrospectively reviewed. The clinicopathologic features, immunohistochemical findings and EWS gene translocation study results were analyzed.</p><p><b>RESULTS</b>The age of patients ranged from 10 to 27 years (mean = 20 years). The sites of involvement included ovary (1 case), uterus (1 case), vulva (2 cases) and vagina (2 cases). The greatest diameter of the tumor ranged from 2 to 10 cm (mean = 5.4 cm). The tumor had nodular appearance and showed grayish-pink fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Histologically, the tumor was composed of malignant small round cells with indistinct cell borders, hyperchromatic nuclei, dense chromatin, tiny nucleoli and scanty cytoplasm. The tumor cells were arranged in sheets or lobules. Homer-Wright rosettes were identified in 1 case. Immunohistochemical study showed that the tumor cells were positive for CD99, FLI-1 and CD56 (6/6). Focal expression of vimentin (5/6), NSE (5/6), nestin (4/6), synaptophysin (4/6), S-100 protein (2/6) and chromogranin A (1/6) was also demonstrated. EWS gene translocation was detected in 5 cases studied. Follow-up information was available in 2 patients (7 and 17 months of follow up, respectively). One of them died of tumor metastasis 17 months after diagnosis. The other patient was still alive.</p><p><b>CONCLUSIONS</b>PNET arising in female genital tract is rare. It mainly involves ovary, uterus, vulva and vagina. Immunohistochemical study using a panel of antibodies and fluorescence in-situ hybridization play an important role in definitive diagnosis of this rare malignancy.</p>

Granulocytic sarcoma: a clinical and pathologic analysis of ten cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and pathological features, differential diagnosis of granulocytic sarcoma.</p><p><b>METHODS</b>The clinical manifestations, histopathological features, immunohistochemistry, treatment and prognosis were analyzed retrospectively in 10 cases of granulocytic sarcoma.</p><p><b>RESULTS</b>The age of patients ranged from 10 to 56 years (means = 35.8 years). The male-to-female ratio was 1.5:1. Histologically, the malignant cells of granulocytic sarcoma grew in a diffuse pattern. The cytoplasm was scanty, with eosinophilic fine granularity in some cells. The nuclei were round or focally irregular, and had finely dispersed chromatin. The mitotic figures were visible. Immunohistochemical stains for MPO, CD43, CD117, CD34 and CD99 were positive.</p><p><b>CONCLUSIONS</b>Granulocytic sarcoma can occur in patients of all ages with a male predominance. The diagnosis of granulocytic sarcoma is assisted by the cytochemical stain for naphthol-ASD-chloroacetate esterase and/or immunophenotypic analyses for MPO, CD43, CD117, CD34, CD99. These stains aid in the distinction of granulocytic sarcoma from: lymphoblastic lymphoma, Burkitt lymphoma, diffuse large B-cell lymphoma, small round cell tumours, particularly in children, and blastic plasmacytoid dendritic cell neoplasm.</p>

Co-expression of CD99/MIC2 and ALK in anaplastic large-cell lymphoma tissues and its significance / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate co-expression of CD99/MIC2 and anaplastic lymphoma kinase (ALK) protein in anaplastic large-cell lymphoma (ALCL) tissues and Karpas 299 cells and its significance.</p><p><b>METHODS</b>Clinical prognoses and ALK protein expressions of 25 cases of ALCL were reviewed retrospectively, the median duration of survival was analyzed for patients with ALK(+) ALCL and ALK(-) ALCL. Histological and immunohistochemical staining were applied to other 25 cases of ALCL and paraffin-embedded tissue from human anaplastic large-cell lymphoma Karpas 299 cells to detect the protein of CD99 and ALK.</p><p><b>RESULTS</b>Of former 25 cases of ALCL, median duration of survival for ALK(+) patients was 59 months, whereas 20 months for ALK(-) patients. The prognosis of ALK(+) group was better than that of ALK(-) group, survival curves of these two groups showed statistically significant (P < 0.05). CD99 was positive in 18 cases (72.0%) while negative in 7 cases (28.0%) of the latter 25 ALCL, ALK was positive in 19 cases (76.0%) while negative in 6 cases (24.0%); Of 19 ALK(+) ALCL, 16 (84.2%) cases co-expressed CD99-ALK; and in 6 ALK(-) ALCL, 2(33.3%) were CD99-ALK double negative, the expression of CD99 protein strongly correlated with that of ALK protein (P < 0.05). ALK and CD99 protein expressed in Karpas 299 cells with diffuse distribution.</p><p><b>CONCLUSIONS</b>CD99 highly expressed in ALCL, and showed high rate of co-expression with ALK. CD99 protein expression could be considered as a helpful diagnostic and prognostic factor of ALCL, especially for ALK(+) ALCL.</p>

Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic and immunohistochemical features of mesenchymal chondrosarcoma.</p><p><b>METHODS</b>The clinical and histologic features of 23 cases of mesenchymal chondrosarcoma were analyzed. Immunohistochemical study was also performed in 14 of the cases.</p><p><b>RESULTS</b>The age of patients ranged from 12 to 47 years. Fourteen of them occurred in males. Thirteen cases involved the bony skeleton and 5 cases affected the soft tissue. The patients presented with pain and/or swelling. Histologically, the tumor consisted of a mixture of undifferentiated small round cells and hyaline cartilage. Transition between the two components was demonstrated and growth plate-like cartilage was observed. Immunohistochemical study showed that the small round cells were positive for Sox9 (14/14), CD99 (12/14), vimentin (6/14), CD56 (4/14), CD57 (4/14), neuron-specific enolase (3/14) and desmin(1/14). They were negative for Coll-II, S-100 protein, epithelial membrane antigen, pan-cytokeratin, synaptophysin, chromogranin A, CD34 and c-erbB2.</p><p><b>CONCLUSIONS</b>Mesenchymal chondrosarcoma is a rare malignant tumor. Thorough histologic examination, when coupled with immunohistochemical findings, is helpful in arriving at a correct diagnosis.</p>

Clinicopathologic features of primary osteosarcoma in elderly patients / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinical manifestations, radiologic findings, pathologic diagnosis and differential diagnosis of primary osteosarcoma in elderly patients.</p><p><b>METHODS</b>Twelve cases of primary osteosarcoma occurring in patients older than 60 years were encountered during the period from 1985 to 2010. The clinical manifestations, radiologic features and pathologic findings were studied and the follow-up data were analyzed.</p><p><b>RESULTS</b>The sites of involvement included long bones (number = 7), ilium (number = 1), craniofacial bones (number = 2) and soft tissue (number = 2). Radiologic examination showed a mixture of osteosclerotic and osteolytic lesions in 10 patients, soft tissue lesions with high-density areas in 2 patients and soft tissue lesions with periosteal reaction in 8 patients. Histologically, most cases showed features of conventional osteosarcoma. There were 2 cases of malignant fibrous histiocytoma-like osteosarcoma, 2 cases of chondroblastic osteosarcoma and 1 case of well-differentiated intraosseous osteosarcoma. Immunohistochemical study played little role in pathologic diagnosis. Ten patients had undergone amputation, including one patient who had received adjuvant chemotherapy beforehand. Nine patients had follow-up information available. Three of them died of lung metastasis and 1 died of cardiovascular disease.</p><p><b>CONCLUSIONS</b>Primary osteosarcoma rarely occurs in elderly patients and can easily be missed. Correlation with clinical, radiologic and histologic features is important for arriving at a correct diagnosis.</p>

Imaging features and clinicopathological manifestations of solitary fibrous tumors / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the imaging features, clinical manifestations and pathological characteristics of solitary fibrous tumors (SFT).</p><p><b>METHODS</b>The clinicopathological manifestations and medical imaging findings were analyzed retrospectively in 27 patients with surgically confirmed SFT.</p><p><b>RESULTS</b>The SFTs originated from different parts of the body, including 18 in the chest, 4 in the abdomen, 1 in the lumboscral area, 3 in the pelvis, and 1 in the left shoulder. Twenty-three cases were found by CT scan, among which there were 16 benign diseases, presented with well-defined round or elliptic margins, with homogeneous attenuation and clearly surrounding; 6 malignant cases with unclear demarcations, invasive surrounding, heterogeneous attenuation due to calcification and/or irregular necrosis, and 1 junctional case with well-defined margins, which was enlarged during follow-up. There were 4 SFTs scanned by MRI with clear margin and homogeneous or heterogeneous signal intensity. All of the 4 cases were isointense or hyperintense to muscle on T1-weighted images, and were hyperintense on the T2-weighted images. All tumors showed heterogeneously intense enhancement with geographic pattern. Immunohistochemical staining showed that CD34-positive was 81.5%, vimentin (100.0%), CD99 (100.0%) and bcl-2 (96.3%), as well as negative CK (100.0%) and S-100 (96.3%).</p><p><b>CONCLUSION</b>The location of SFT is varying. Though its clinical manifestations vary, the diagnosis is depended on pathology and immunohistochemistry. There are certain specific features related to SFTs on CT or MRI. These imaging techniques may serve to provide helpful information as to the location and vicinal anatomic structure of the tumor, which is of substantial importance for planning surgery.</p>

Expression of FLI-1 and analysis of prognostic factors in primitive neuroectodermal tumor / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To observe the expression of FLI-1 in primitive neuroectodermal tumors (PNET), explore the value of immunohistochemical staining of FLI-1 in combination with other neural markers in diagnosis of PNET, and analyze the prognostic factors in PNET patients.</p><p><b>METHODS</b>35 cases of PNET, of which 33 cases with complete clinical data, were included in this study. Immmunohistochemistry (The En Vision method) was applied to detect the expression of FLI-1, CD99, Syn, NSE, S-100, NF, Vim in the tumor tissues. The clinicopathological data of 33 cases were analyzed by Cox regression.</p><p><b>RESULTS</b>The positive expression rate of FLI-1 were 51.4% and that of CD99 was 88.6%. The sensitivity of FLI-1 combined with CD99 was up to 100%. The positive rates of Vim, Syn, NSE, s-100 and NF were 91.4%, 48.6%, 45.7%, 22.9% and 0, respectively. Cox regression analysis showed that the impact of primary location and treatment modality were of statistical significance (P < 0.05), but the age, sex, stage or size of tumors did not (P > 0.05).</p><p><b>CONCLUSION</b>Immunohistochemical detection of FLI-1 and neural markers is a preferred method for clinical diagnosis of PNET. The main factors affecting the prognosis are the primary location of PNET and treatment modality.</p>

Clinicopathologic analysis of 4 cases of primary renal synovial sarcoma / 癌症

<p><b>BACKGROUND AND OBJECTIVE</b>Primary renal synovial sarcoma is rare and might be misdiagnosed as another renal tumor. This study demonstrates the clinicopathologic and immunohistochemical features, differential diagnosis, and prognosis of such tumors.</p><p><b>METHODS</b>Histologic slides and clinical data were reviewed for 4 patients with primary renal synovial sarcoma and immunohistochemical staining was performed. Molecular analysis was performed on 2 cases to demonstrate the presence of the SYT-SSX gene fusion transcripts by reverse transcriptase polymerase chain reaction (RT-PCR).</p><p><b>RESULTS</b>The patients were 2 women and 2 men aged from 32 to 48 years. The tumors were 10.0-15.0 cm in diameter, grey-white and solid, and hemorrhage or necrosis was observed. Microscopically, the tumors consisted of mitotically active, monomorphic plump spindle cells with indistinct cell borders growing in short, intersecting fascicles. Hypocellular myxoid areas and a prominent hemangiopericytomatous pattern were present in all cases. The average mitotic rate was 5-8 mitoses/10 high-power fields. Hemorrhage and tumor necrosis were easily found. Scattered small cysts lined with flat, cuboidal, or hobnailed epithelia were found in 3 cases. Tumor cells are immunoreactive for Vimentin (4/4), Bcl-2 (4/4), CD99 (4/4), and CD56 (3/4), and focally for EMA (3/4) and Cytokeratin (3/4). SYT-SSX1 gene fusion was detected in the 2 cases in which RT-PCR analysis was performed. One patient had tumor metastasis to the lung 6 months after surgery and died 5 months later. Multiple metastasis to the liver occurred in one patient and the patient died 13 months after the initial surgery. The other 2 patients had tumors recur at 8 and 15 months and died at 18 and 21 months, respectively, after the initial operation.</p><p><b>CONCLUSION</b>Primary renal synovial sarcoma is rare, with poor prognosis, characterized by SYT-SSX gene fusion, and needs to be differentiated from other renal sarcomas.</p>

Extraskeletal Ewing's sarcoma: a report of 18 cases and literature review / 癌症

<p><b>BACKGROUND AND OBJECTIVE</b>Extraskeletal Ewing's sarcoma (EES) is a rare, rapidly growing, round-cell, malignant tumor that can develop in the soft tissues at any location. This study was to analyze the clinical features, diagnosis and treatment of EES.</p><p><b>METHODS</b>Clinical data of 18 patients with EES, treated at between Cancer Center of Sun Yat-sen University between 1995 and 2007, were analyzed.</p><p><b>RESULTS</b>Of the 18 patients, 13 were male and 8 were female, aged from 8 months to 60 years. Twelve (66.7%) patients were between 5-25 years of age. Eight (44.4%) patients had tumors originated from low extremities.Sixteen patients had masses at their first visit. Sixteen patients were treated by the combined modality therapy, and 2 patients were treated by the single modality therapy. The 1-, 3- and 5- year actuarial survival rates were 82.4%, 64.2% and 32.1%, respectively. The presence of metastatic disease at the time of diagnosis and the mode of treatment were prognostic factors.</p><p><b>CONCLUSIONS</b>EES is common in adolescent. It often manifests as a localized mass. The combined modality therapy is recommended for this disease. The presence of metastatic disease at the time of diagnosis and the mode of treatment are prognostic factors.</p>

Clinicopathologic study of juvenile granulosa cell tumor of ovary / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, diagnostic criteria and prognostic parameters of juvenile granulosa cell tumor of ovary.</p><p><b>METHODS</b>The clinical and pathologic findings of 7 cases of juvenile granulosa cell tumor were retrospectively reviewed. Immunohistochemical study was carried out in 6 of these cases. The follow-up data were also analyzed.</p><p><b>RESULTS</b>The mean age of the patients was 24 years (range=6 to 53 years). Four patients presented with hormonal disturbance, while 3 patients presented with abdominal pain or swelling. Six patients underwent unilateral salpingo-oophorectomy. Six cases were in stage IA and the remaining case in stage IC. Follow-up information was available in 6 patients and the duration of follow up ranged from 1 to 10 years (mean=4.3 years). Five patients remained healthy, with no evidence of tumor recurrence. One patient died of tumor metastasis one year after the diagnosis. Gross examination showed that the tumor masses ranged from 7 to 20 cm in the greatest dimension (average=13.4 cm). Four of the 7 tumors were mixed solid-cystic in appearance and 2 cases were unilocular cystic in nature. Microscopic examination showed diffuse atypical follicular structures formed by granulosa cells. The granulosa cells contained round hyperchromatic nuclei, without nuclear grooves or Call-Exner body formation (6/7). In one of the cases studied, minor foci resembling adult granulosa cell tumor were also demonstrated. The degree of cellular atypia varied (3 cases with severe atypia, 1 case with moderate atypia and 3 cases with mild atypia). The mitotic count ranged from 1 to more than 5 per 10 high-power fields. Immunohistochemical study showed diffuse positivity for vimentin (6/6). The staining for cytokeratin (AE1/AE3) and calretinin was negative. Four cases expressed CD99 and 1 case was positive for inhibin.</p><p><b>CONCLUSIONS</b>Juvenile granulosa cell tumor is characterized by the presence of diffuse atypical follicular structures formed by small round cells, without nuclear grooves or Call-Exner bodies. Rare cases contain minor foci of adult granulosa cell tumor. They can be unilocular cystic in nature. The degree of nuclear atypia, mitotic activity and size of the tumor vary and do not correlate with the risk of recurrence and aggressive biologic behavior.</p>

Superficial acral fibromyxoma of finger: report of a case with review of literature / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic characteristics, immunophenotype and differential diagnosis of superficial acral fibromyxoma (SAF).</p><p><b>METHODS</b>The clinical, pathologic and immunohistochemical features of a case of SAF occurring in left middle finger was studied, with review of literature.</p><p><b>RESULTS</b>The patient was a 62-year-old male who presented with a solitary painful nodule located in the distal aspect of his left middle finger. The nodule lied close to the nail bed and deep to the underlying periosteum. Grossly, the tumor was poorly circumscribed, measured 2 cm in greatest dimension and had a greyish-white cut surface and rubbery consistency. On low-power examination, the tumor was centred in the dermis and displayed a vague lobular pattern. The tumor cells were spindled to stellate in shape and associated with myxoid matrix. Focal fascicular or loose storiform patterns were also noted. A delicate vascular network was identified in the myxoid stroma. Mast cells were readily observed. On high-power examination, the tumor cells were relatively bland-looking and showed at most a mild degree of nuclear atypia. Mitotic figures were rare and coagulative tumor necrosis was absent. Immunohistochemical study showed that the tumor cells were positive for vimentin, CD34 and CD99. Focal staining for CD10 was also demonstrated. Other immunomarkers including actins, desmin and epithelial membrane antigen were negative.</p><p><b>CONCLUSIONS</b>SAF is a distinctive soft tissue tumor occurring mainly in the digits of adults. Awareness of this entity is helpful in distinguishing SAF from other myxoid soft tissue tumors occurring there. Complete excision with clear resection margins is the mainstay of treatment.</p>

Construction of mic2/CD99 gene vector and its transfection in Hodgkin lymphoma L428 cell line / 南方医科大学学报

<p><b>OBJECTIVE</b>To construct a eukaryotic expression vector of CD99 gene for transfection into Hodgkin lymphoma L428 cells.</p><p><b>METHODS</b>The full-length cDNA of CD99 gene was amplified from Jurkat cells by RT-PCR and cloned into the pcDNA3.1(+) vector and transfected into L428 cell line using Lipofextamine 2000. The sequence of CD99 mRNA in the transfected cells was confirmed by restriction endonuclease digestion and DNA sequencing, and the expression of CD99 protein was identified using immunocytochemistry.</p><p><b>RESULTS</b>A gene fragment of 558 bp was amplified from the transfected cells and the sequence was verified by DNA sequencing. Immunocytochemistry identified the presence of CD99 expression in the transfected cells.</p><p><b>CONCLUSION</b>A eukaryotic expression vector pcDNA3.1(+)-CD99 is successfully constructed and stably expressed in L428 cell line.</p>

Clinicopathologic characteristics of hemangiopericytoma/solitary fibrous tumor with giant cells / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the pathological characteristics, diagnosis and differential diagnoses of hemangiopericytoma-solitary fibrous tumor with giant cells.</p><p><b>METHODS</b>Pathological characteristics of seven cases of orbital and extraorbital hemangiopericytoma-solitary fibrous tumors with giant cells were evaluated by HE and immunohistochemistry (EnVision method).</p><p><b>RESULTS</b>Two cases were located in the orbit, one of which had recurred. Five cases were located in the extraorbital regions. Histologically, the tumors were well-circumscribed and composed of non-atypical, round to spindle cells with collagen deposition in the stroma. The tumors had prominent vasculatures and in areas, pseudovascular spaces lined by multinucleated giant cells lining which were also present in the stroma. Immunohistochemically, both neoplastic cells and multinucleate giant cells expressed CD34. Seven patients underwent tumor excision and were well and without tumor recurrence upon the clinical follow-up.</p><p><b>CONCLUSIONS</b>Hemangiopericytoma-solitary fibrous tumor with giant cells is an intermediate soft tissue tumor. It typically involves the orbital or extraorbital regions. Histologically, the tumor should be distinguished from giant cell fibroblastoma, pleomorphic hyalinzing angiectatic tumor of soft part and angiomatoid fibrous histiocytoma.</p>

Small cell variant of peripheral T-cell lymphoma, not otherwise specified: a clinicopathologic and immunophenotypic analysis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of small cell variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS).</p><p><b>METHODS</b>The clinicopathologic features of 5 cases of small cell variant of PTCL, NOS were retrospectively reviewed, with immunohistochemical study, T-cell receptor (TCR) gene rearrangement analysis and evaluation for Epstein-Barr virus (EBV) status.</p><p><b>RESULTS</b>All the 5 patients were males. The mean age was 52.6 years. The median duration before diagnosis was 1 month. Clinically, 3 patients presented in stage IV and 2 in stage III. Four of them had generalized lymphadenopathy and splenomegaly. Hepatomegaly and massive effusion were found in 1 and 2 cases, respectively. Marrow involvement was detected in 3 of the 4 patients with bone marrow biopsy performed and one of them also accompanied by lymphocytosis. Histologically, the involved lymph nodes showed partial or complete effacement of nodal architecture and replacement by a monomorphous population of small lymphoid cells. Scanty large lymphoid cells were also identified in 4 cases. Increase in number of blood vessels was noticed in two of them as well. Immunohistochemically, the lymphoma cells in all cases expressed two or more of the T-cell markers and CD43. The staining for CD20, TdT, CD56 and granzyme B was negative. CD99 expression was noted in 3 of the 4 cases. The Ki-67 index ranged from 5% to 15%. Clonal TCRgamma gene rearrangement was detected in the 4 cases studied and one of them also showed TCRbeta gene rearrangement. In-situ hybridization for EBV-encoded RNA was negative in the 4 cases studied. Follow up information was available in 3 of the 5 cases. All of the 3 patients died of the disease, with an average survival of 21.7 months.</p><p><b>CONCLUSION</b>Small cell variant of PTCL, NOS represents a rare disease entity which often presents in advanced tumor stage and carries a poor prognosis.</p>

Lymphoblastic lymphoma and acute lymphoblastic leukemia: a clinicopathologic, immunophenotypic and prognostic study in 153 Chinese patients / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, immunohistochemical findings and prognosis of precursor lymphoblastic lymphoma/acute lymphoblastic leukemia (LBL/ALL).</p><p><b>METHODS</b>One hundred and fifty-three cases of LBL/ALL were retrospectively analyzed. Immunohistochemical study was carried out. The pathologic findings were correlated with Ann Arbor tumor stage, Ki-67 index, other clinical parameters (including mediastinum/bone marrow involvement, hepato-splenomegaly, age and gender of the patients) and the survival data.</p><p><b>RESULTS</b>Staining for TdT and CD99 was positive in 79.1% (121/153 cases) and 96.3% (131/136 cases), respectively. The cases were categorized into three groups according to the immunohistochemical findings, as follows: precursor T-cell, precursor B-cell and undefined. T-LBL/ALL accounted for 69.3% (106/153 cases) of all of the cases. The male-to-female ratio was 2.4:1 (including 75 males and 31 females). The median age at diagnosis was 17.5 years (ranged from 2 years to 68 years). Ninety-two patients (86.8%) presented with peripheral lymphadenopathy and 59 of them (55.7%) had mediastinal masses. Ninety-one cases (85.8%) were in stage III or IV at diagnosis. The 1-year and 5-year survival rates in patients with T-LBL/ALL were 36.1% and 8.1%, respectively. Patients older than 25 years and those presented in stage III or IV suggested a poor prognosis (P = 0.049 and 0.001, respectively). On the other hand, 29 of the 153 cases (19.0%) belonged to B-LBL/ALL. The median age of the patients was 14 years (ranged from 9 months to 75 years). The male-to-female ratio was 1.6:1 (including 18 males and 11 females). Seventeen patients (58.6%) presented with peripheral lymphadenopathy and 13 of them (44.8%) had involvement of bone marrow or peripheral blood. Mediastinal involvement was found only in 5 cases (17.2%). Twenty-one patients (72.4%) were in stage III or IV at diagnosis. The 1-year and 5-year survival rates were 53.3% and 36.7%, respectively. The remaining 11.7% cases (18/153 cases) were categorized as undefined type, with a negative staining for the following immuno-markers including: CD3ε/CD3, CD45RO, CD79a, CD20, MPO, CD5, CD56, cyclin D1, cytokeratin, neuron-specific enolase, chromogranin A and synaptophysin. The median age of the patients was 15.5 years (ranged from 4 to 53 years). The male-to-female ratio was 2.6:1 (including 13 males and 5 females). The percentage of T-LBL/ALL patients with mediastinal masses were significantly higher than that of B-LBL/ALL cases (P = 0.0003). There was no significant difference in prognostic parameters of T-LBL/ALL and B-LBL/ALL (P = 0.07). The difference in median survival time however was statistically significant (6.0 months +/- 1.1 months versus 15.0 months +/- 7.0 months).</p><p><b>CONCLUSIONS</b>Both TdT and CD99 are useful markers for the diagnosis of precursor lymphoblastic malignancy. T-LBL/ALL predominantly affects children or adolescent males and frequently presents with lymphadenopathy and mediastinal masses, whereas B-LBL/ALL are often accompanied by bone marrow and peripheral blood involvement. In general, T-LBL/ALL carries a poor prognosis. The prognostic criteria include age of older than 25 years and a classification of stage III or IV disease.</p>