A case report of fungal infection associated acute fibrinous and organizing pneumonitis.

BACKGROUND: Acute fibrinous and organizing pneumonitis (AFOP) is an uncommon variant of acute lung injury, characterized by intra-alveolar fibrin and organizing pneumonia. Proposed etiologies include connective tissue diseases, infections, occupational exposure, drug reactions, and autoimmune disease. Here we present a rare case of fungal infection associated AFOP in patient with diabetes mellitus (DM) and review the relevant literature. CASE PRESENTATION: A 67-year-old man complained of cough, fever, dyspnea and hemoptysis. Patient experienced a rapidly progressive course exhibit diffuse predominant consolidation, ground glass opacities, and multifocal parenchymal abnormalities on chest computed tomography (CT). Antibacterial, antifungal, and antiviral treatments were ineffective. A CT-guided percutaneous lung biopsy was performed. Histologically, the predominant findings were as follows: alveolar spaces filled with fibrin and organizing loose connective tissues involving 70% of the observed region, pulmonary interstitial fibrosis, and small abscesses and epithelioid cell granuloma in the focal area. Result of periodic acid-silver methenamine stain was positive. The fungal pathogen from the sputum culture was identified as P. citrinum repeatedly over 3 times. Patient was diagnosed with DM during hospitalization. Corticosteroids combined with an antifungal therapy were effective. Follow-up for 4 months showed complete radiological resolution. CONCLUSIONS: As this common "contaminant" can behave as a pathogen in the immunocompromised host, both clinicians and microbiologists should consider the presence of a serious and potentially fatal fungal infection on isolation of P. citrinum. Based on this case, it could be speculated that AFOP may be associated with fungal infection including P. citrinum.

[Clinicopathologic features of testicular seminoma with syncytiotrophoblastic cells: Analysis of 3 cases and review of the literature].

OBJECTIVE: To investigate the clinical and histopathologic features of testicular seminoma with syncytoplasmic trophoblastic components. METHODS: Using light microscopic staining, we analyzed the clinical and histopathologic characteristics, diagnosis, differential diagnosis and prognosis of 3 cases of testicular seminoma with syncytoplasmic trophoblastic components, and reviewed the relevant literature. RESULTS: All the 3 cases were typical seminoma with syncytiotrophoblastic giant cells. Immunohistochemistry showed strong expressions of CD117 OCT-4, SALL4 and PLAP in diffuse tumor cells, and that of hCG in syncytiotrophoblastic cells. Continuous monitoring and consultation exhibited normal levels of serum ß-hCG in all the cases after postoperative chemotherapy. CONCLUSIONS: Testicular seminoma with syncytiotrophoblastic giant cells and increased serum ß-hCG is a rare subtype, which occurs mostly in young people, sensitive to chemotherapy postoperatively and with a relatively good prognosis.

[Clinicopathological characterization of non-Hodgkin lymphoma of the prostate].

OBJECTIVE: To study the clinicopathological characteristics of non-Hodgkin lymphoma (NHL) of the prostate. METHODS: We collected the clinical data on 6 cases of NHL of the prostate pathologically confirmed between 2001 and 2017. The patients were aged 49－76 (median 62) years old, with the main clinical manifestations of painless swelling of the prostate and lower urinary tract obstruction. We analyzed the clinical features and the results of histological detection, immunohistochemical staining and B-cell gene rearrangement assay, and explored the clinicopathological characteristics and differential diagnosis of the disease based on the relevant literature. RESULTS: Histological detection revealed diffuse large B-cell lymphoma (DLBCL) in 4 cases (66.7%), B-lymphoblastic lymphoma (B-LBL) in 1 (16.7%), and Burkitt lymphoma (BL) in another (16.7%). DLBCL was histologically characterized by diffuse oval or round medium-to-large-sized lyphoid cells with an infiltrative growth pattern, B-LBL by monotonous small-to-medium-sized lymphoid cells with prominet mitosis and apoptosis, and BL by diffuse and monotonous medium-sized neoplastic cells with round or oval nuclei, an infiltrative growth pattern, scanty cytoplasm and visible mitosis. One of the DLBCL patients received 5 doses of R-CHOP chemotherapy and has been followed up to the present time, while the other 3 were lost to follow-up; the B-LBL patient died at 1 month after diagnosis; and the BL patient gave up treatment. CONCLUSIONS: Non-Hodgkin's lymphoma of the prostate mostly presents as diffuse large B-cell lymphoma, and its diagnosis depends on immunohistochemistry and related molecular detection as well as its clinical and histopathological manifestations.

[Primary testicular diffuse large B-cell lymphoma: A clinicopathological analysis].

OBJECTIVE: To investigate the clinicopathological features, immunophenotype and treatment of primary testicular diffuse large B-cell lymphoma (DLBCL). METHODS: We retrospectively analyzed the pathomorphological characteristics and immunohistochemical markers of 23 cases of primary testicular DLBCL as well as their clinicopathological features with a review of the relevant literature. The patients were aged 48－76 (mean 61.4) years, 82.6% over 50 years, and all clinically presented with painless progressive unilateral testicular swelling, 9 cases in the left and the other 14 in the right testis. RESULTS: Histologically, the lymphomas were composed of large atypical cells with prominent karyokinesis and diffusely infiltrated the testicular parenchyma. The neoplastic cells were positive for B-cell markers. Five of the patients were followed up for 2 to 32 months, of whom 4 survived and 1 died at 9 months. CONCLUSIONS: Primary testicular DLBCL is a rare tumor with an invasive biological behavior, mostly found in elderly males and easily misdiagnosed or missed in diagnosis. Histopathology plays a key role and immunohistochemical markers are of high value in the definite diagnosis and differential diagnosis of the tumor.

Thymidine kinase 1 expression in ovarian serous adenocarcinoma is superior to Ki-67: A new prognostic biomarker.

Cancer is a disease with abnormally proliferating cells and therefore proliferation rate is an important index for assessing tumour growth. Ki-67 is a commonly used proliferation marker considered to be an unfavourable prognostic marker in some tumors, while Thymidine kinase 1 (TK1) is an interesting proliferation marker because its levels are highly dependent on the growth stage of cells. To define the immunohistochemistry (IHC) expression of the TK1 in patients with ovarian serous adenocarcinoma and establish its potential role as a new biomarker for progressive disease, we analyzed the expression patterns of TK1 and Ki-67 in 109 patients with ovarian serous adenocarcinoma. TK1 and Ki-67 expression both showed a statistically significant correlation to MD Anderson Cancer Center (MDACC) grade, but not to age, tumour size, lymph node metastasis or pathological TNM (pTNM) stages. TK1 expression, MDACC grades, pathological stages and lymph node metastasis correlate to relapse incident rate and overall survival, but Ki-67 does not. Although TK1 expression, MDACC grade, pTNM stage and lymph node metastasis significantly correlate to relapse in the Cox univariate analysis, in the multivariate Cox analysis only TK1 expression and lymph node metastasis were independent prognostic factors. The overall survival also correlated significantly to TK1 expression, MDACC grade, pTNM stage and lymph node metastasis in the Cox univariate analysis. However, only the pTNM stage was found to be an independent prognostic factor for survival in the Cox multivariate analysis. Therefore, though TK1 expression was an independent prognostic factor for relapse, but not for survival, TK1 is a more informative expression than Ki-67 for LI, relapse and overall survival rates. Thus, when TK1 is combined with MDACC grading, pTNM staging and lymph node metastasis, IHC determination of TK1 expression may improve the overall prediction of prognosis in patients with ovarian cancer.

DCN deficiency promotes renal cell carcinoma growth and metastasis through downregulation of P21 and E-cadherin.

Decorin (DCN), as an important component of the extracellular matrix (ECM), is a small leucine-rich proteoglycan and synthesized by fibroblasts. Although DCN is dysregulated in numerous cancer types, limited data are available on the expression level and important role of DCN proteins in renal cell carcinoma (RCC). In our study, we examined the expression patterns of DCN messenger RNA (mRNA) in RCCs through the Oncomine database and DCN protein in 94 RCC specimens by immunohistochemistry (IHC). The results revealed that DCN expression was decreased in cancerous tissues compared to adjacent noncancerous tissues and was highly correlated to tumor size. Then, via gain-of-function analyses, DCN overexpression could inhibit RCC cell proliferation and metastasis in vitro and vivo. At the mechanism level, we found that an ectopic expression of DCN significantly upregulated P21 and E-cadherin expression. Altogether, these results revealed that DCN is a tumor suppressor in RCC, and it could serve as a potential therapeutic target in patients with RCC.

[Alveolar soft part sarcoma: a clinicopathologic analysis of 48 cases].

OBJECTIVE: To study the clinicopathologic features and differential diagnosis of alveolar soft part sarcoma (ASPS). METHODS: The clinical data and pathologic features of 48 cases of ASPS were evaluated. Immunohistochemical study, PAS staining and fluorescence in-situ hybridization (FISH) were carried out in selected examples. Relevant literature was reviewed. RESULTS: Amongst the 48 cases studied, there were 17 males and 31 females, with male-to-female ratio of 1.0∶1.8. The age of patients ranged from 2 to 60 years (median=26 years). The tumor was most commonly located in deep soft tissue, especially that of lower extremities. Histologically, the tumor cells were arranged in alveolar or solid patterns and separated by sinusoidal vessels. They were large and contained abundant eosinophilic granules or crystals in cytoplasm. The nuclei were round to polygonal and vesicular, often with prominent nucleoli. Intravascular tumor extension was common. Some cases showed necrosis, hemorrhage and cystic changes. Immunohistochemical study showed that the tumor cells were positive for TFE3 (100%, 33/33). FISH assay was carried out in 4 cases and all of them had TFE3-ASPL gene fusion. CONCLUSIONS: ASPS is a rare malignant neoplasm, often occurs in young patients. TFE3 is a useful immunohistochemical marker for diagnosis. The diagnosis is further confirmed by other markers.

[Diagnostic value of immunohistochemistry and FISH for chromosome 12p in type â ¡ testicular germ cell tumors].

OBJECTIVE: To study the pathological morphology, immunohistochemical characteristics, and molecular changes of type Ⅱ testicular germ cell tumors (TGCT) and investigate the possible value of immunohistochemistry and fluorescence in situ hybridization (FISH) in the diagnosis of TGCT. METHODS: We collected for this study 97 cases of TGCT, including 75 cases of seminoma, 17 cases of embryonal carcinoma, 11 cases of yolk sac tumor, 16 cases of mature teratoma, 3 cases of immature teratoma, and 1 case of epidermoid cyst, in which normal testicular tissue was found in 20 and non-TGCT in 6. We detected the expressions of different antibodies in various subtypes of TGCT by immunohistochemistry and determined the rate of chromosome 12p abnormality using FISH. RESULTS: The immunophenotypes varied with different subtypes of TGCT. SALL4 and PLAP exhibited high sensitivity in all histological subtypes. CD117 and OCT4 showed strongly positive expressions in invasive seminoma and germ cell neoplasia in situ (GCNIS) but not in normal seminiferous tubules. GPC3 was significantly expressed in the yolk sac tumor, superior to GATA3 and AFP in both range and intensity. CKpan, OCT4, and CD30 were extensively expressed in embryonal carcinoma, while HCG expressed in choriocarcinoma. The positivity rate of isochromosome 12p and 12p amplification in TGCT was 96.7% (29/30). CONCLUSIONS: The majority of TGCT can be diagnosed by histological observation, but immunohistochemical staining is crucial for more accurate subtypes and valuable for selection of individualized treatment options and evaluation of prognosis. Chromosome 12p abnormality is a specific molecular alteration in type Ⅱ TGCT, which is useful for ruling out other lesions.

[Embryonal rhabdomyosarcoma in the male reproductive system: A clinicopathological analysis].

OBJECTIVE: To investigate the pathological characteristics, diagnosis, and differential diagnosis of embryonal rhabdomyosarcoma (ERMS) in the male reproductive system. METHODS: We obtained the clinicopathological features, immunophenotypes, and electron microscopic findings of 11 male patients with ERMS in the reproductive system from 2000 to 2015, analyzed the data, and reviewed relevant literature. RESULTS: ERMS developed in these patients at a median age of 17 (9－58) years, 3 cases in the testis, 4 in the scrotum, 1 in the epididymis, and 3 in the prostate. ERMS presented no clinical specificity, which made it difficult to be differentiated from inflammatory and other benign lesions. Microscopically, the tumor cells were arranged in a diffuse or fascicular distribution and mainly composed of short spindle-like, round, or irregularly shaped cells with nuclear hyperchromatism, the cytoplasm strongly eosinophilic, with differentiation of the striated muscle. Some of the cells were naively differentiated or tennis racket-shaped and some exhibited vacuolar degeneration in the cytoplasm. The nuclei were round or short spindle-shaped with visible nucleoli and mitoses. Immunohistochemically, the tumor cells were positive for Myogenin (5/6), Desmin (11/11), MyoD1 (8/9), and Myosin (1/2). Electron microscopy revealed early myofibrils in the cytoplasm of the tumor cells. CONCLUSIONS: ERMS is a rare and highly malignant tumor characterized by local invasion and early metastasis and apt to develop in the reproductive system of young males. The diagnosis of the malignancy is mainly based on its histopathological and immunohistochemical manifestations, combined with electron microscopy when necessary. Early surgical resection in combination with radio- and chemotherapy is recommended for its treatment, which could reduce the recurrence of the tumor and improve the survival of the patients.

Frequent co-inactivation of the SWI/SNF subunits SMARCB1, SMARCA2 and PBRM1 in malignant rhabdoid tumours.

AIMS: Malignant rhabdoid tumours (MRTs) are highly aggressive malignancies of early infancy characterized by inactivation of SMARCB1, a core member of the SWI/SNF chromatin-remodelling complex. The aim of this study was to explore the status of multiple key subunits of the SWI/SNF complex in MRTs. METHODS AND RESULTS: We screened the key subunits of the SWI/SNF complex, including SMARCB1, SMARCA2, PBRM1, SMARCA4, and ARID1A, in four MRTs by immunohistochemistry, sequencing, and fluorescence in-situ hybridization (FISH). Complete loss of SMARCB1, SMARCA2 and PBRM1 expression and corresponding mutations in the same genes were observed in all cases. The mutations included seven missense, three same-sense, four frameshift and two truncating mutations. FISH revealed heterozygous deletion of SMARCB1 in one case, and monoploidy of chromosome 22, which harbours SMARCB1, in another case. Furthermore, trisomy of chromosome 9, which harbours SMARCA2, was observed in two cases. Abnormality of PBRM1 was not found in any case. CONCLUSIONS: We report, for the first time, co-inactivation and frequent mutations of SMARCB1, SMARCA2 and PBRM1 in MRTs. Multiple subunit abnormalities of the SWI/SNF complex potentially act together to contribute to the tumorigenesis of MRTs, which provides unique insights into this disease.

[ALK gene fusion associated non-small cell lung cancer: automated immunostainer detection and clinicopathologic perspectives].

OBJECTIVE: To explore the automated immunostainer screening anaplastic lymphoma kinase (ALK) gene fusion non-small cell lung cancer (NSCLC) and clinicopathological characteristics of the molecular subtype lung cancers. Methods Five hundred and sixty-six cases of NSCLC were collected over a 16 month period. The test for ALK was performed by Ventana automated immunostainer with anti-ALK D5F3. The histological features, treatment and outcome of patients were assessed. Results Thirty-eight cases (6.7%, 38/566) of NSCLC showed ALK gene fusion. The frequency of ALK gene fusion was higher in male (7.1%, 25/350) than that in female (6.0%, 13/216) patients, but not achieving statistical significance (chi2 = 0.270, P = 0.604). ALK + NSCLC was more significantly more frequent in patients < or = 60 years (9.9%, 28/282) than >60 years (3.5% , 10/284) of age. Histologically, the ALK + NSCLCs were mostly adenocarcinoma (81.6%, 31/38) , among which eighteen cases were solid predominant subtype with mucin production; nine cases were acinar predominant subtype; one case was papillary predominant subtype and three cases were invasive mucinous adenocarcinoma. The ALK + non-adenocarcinoma included three cases of squamous cell carcinoma, three cases of adenosquamous carcinoma and one case of pleomorphic carcinoma. Among the ALK + NSCLC patients, the number of non/light cigarette smokers (86. 8% , 33/38) was more than that of heavy smokers. Twenty-nine cases were stages III and IV; twenty-nine cases showed lymph node metastasis; twenty cases showed metastases mostly to brain and bone; and one case showed EGFR gene mutation coexisting with ALK gene fusion. Twelve of fifteen patients received crizotinib therapy and remained stable. Conclusions NSCLC with ALK gene rearrangement shows distinctive clinical and histological features. Ventana-IHC may he a feasible and valid technique for detection of ALK rearrangement in NSCLC.

[Papillary cystadenoma of the epididymis: a report of 2 cases and review of the literature].

OBJECTIVE: To study the clinicopathological characteristics of papillary cystadenoma of the epididymis. METHODS: Using routine pathology and immunohistochemistry, we observed the surgically obtained samples from 2 cases of papillary cystadenoma of the epididymis, analyzed their pathological features and clinical presentations, and reviewed the related literature. RESULTS: The 2 patients were both adult males. The tumors typically manifested as painless swelling in the epididymis, with occasionally dull pain and tenesmus in 1 of the cases. Pathologically, the lesions exhibited three morphological features, i. e., dilated ducts and small cysts surrounded by fibrous connective tissue, adenoid papillary hyperplasia into the cysts embraced by fibrovascular stroma, and acidophil substance present in the cysts. Immunohistochemistry showed that the tumors were strongly positive for CK8/18, CK7, and EMA, but negative for CK20, CEA, MC, Calretenin, P53, P63, SMA, VHL, and CD10, with the positive rate of Ki-67 <1%. Follow-up visits revealed good prognosis in both cases. CONCLUSION: Papillary cystadenoma of the epididymis is a rare benign tumor in the male urogenital system, which may be accompanied by the VHL syndrome. Surgery is the first choice for its treatment.

Loss of BRM expression is a frequently observed event in poorly differentiated clear cell renal cell carcinoma.

AIMS: The aim of this study was to examine the status of Brahma (BRM), a key SWI/SNF complex subunit, in clear cell renal cell carcinomas (RCCs), and to analyse the histopathology, immunophenotype, molecular features and prognosis of the BRM-negative cases. METHODS AND RESULTS: We identified 19 cases of grade 4 tumours lacking BRM expression among 625 clear cell RCCs. All 19 cases exhibited features of poor differentiation: 13 showed pure poorly differentiated morphology, while six were composite tumours with an admixed typical low-grade component. Besides negative BRM expression, the immunophenotype of these cases was similar to clear cell RCC. VHL gene mutations were identified in nine of the 19 patients (47%). Chromosome 3p deletion was detected in 11 of 13 poorly differentiated RCCs and both areas of five of five composite tumours. All poorly differentiated tumour areas showed polysomy of chromosome 3. No losses or gains of chromosome 3 were observed in low-grade tumour areas of five of five composite RCCs. CONCLUSIONS: We have shown that loss of BRM expression is a common feature among poorly differentiated tumours in clear cell RCCs. We hypothesize that loss of BRM expression is involved in tumor de-differentiation in clear cell RCCs and may play an important role during tumour progression.

Abdominopelvic tuberculosis mimicking advanced ovarian cancer and pelvic inflammatory disease: a series of 28 female cases.

OBJECTIVE: To present and discuss 28 female cases with abdominopelvic tuberculosis (TB) and abnormal CA125 levels to better distinguish this disease from advanced ovarian cancer (AOC) and pelvic inflammatory disease (PID). Abdominopelvic tuberculosis (APTB) is one of the extrapulmonary tuberculosis (TB) sites, usually misdiagnosed as AOC and PID and then has to undergo surgery. However, the treatment of APTB is totally based on medical therapy other than surgery except biopsy. This article aims to present and discuss 28 female APTB cases with abnormal CA125 levels to better distinguish this disease from AOC and PID so as to find out non-invasive APTB diagnosis methods. METHODS: 28 APTB patients diagnosed between January 2000 and January 2010 in our gynecologic department of Nanjing Jinling hospital were reviewed retrospectively and compared with AOC and PID. RESULTS: The mean age was 38.24 ± 11 (range 15-64) years. Elevated levels of serum CA125 were determined in all 28 patients (100%). Other common findings were ascites in 20 (71.43%, 20/28), pelvic mass in 21(75%, 21/28), slight fever with night sweat in 13 (46.43%, 13/28), cough and pleural effusion in nine (32.14 %, 9/28), high fever more than 39 °C combined with abdominal pain and elevated white blood count in five (17.86%, 5/28), weight loss more than 5 kg at admission in six (21.43%, 6/28). Diagnoses were made based on biopsy from laparotomy in 14 (50%) patients, from laparoscopy in nine (32.14%), from diagnostic curettage because of primary infertility in two (7.14%), and only from clinical suspicion in three patients. Histopathology revealed that caseating granulomatous lesions were seen in 25 patients, positive anti-acid staining in 11 patients. Totally 26 patients completed anti-TB therapy successfully and were cured, two patients died of the disease because of long-term immune inhibitor used. CONCLUSION: Although it is difficult to exactly distinguish APTB from AOC and PID without operation, it is important because the treatment of APTB is totally based on medical therapy other than surgery. Some difference may be found out if clinical manifestation, physical examination, laboratory tests and imaging findings are carefully analyzed to avoid unnecessary extensive surgery and improve the prognosis.

[Aggressive B-cell lymphomas of gastrointestinal tract: a clinicopathologic analysis of 54 cases].

OBJECTIVE: To study the histological features, diagnosis, differential diagnoses of aggressive B-cell lymphomas of the gastrointestinal tract and to correlate clinical prognosis with pathologic parameters and immunophenotypes with an emphasis on c-myc, Tcl-1 and CD38 expression and their values in predicting the status of c-myc gene translocation. METHODS: Fifty-four cases of aggressive B-cell lymphomas of the gastrointestinal tract with complete clinical and pathologic data were retrospectively collected. The clinical data, histologic and immunohistochemical findings and follow-up results were analyzed. Predictive immunohistochemical stains including c-myc, Tcl-1 and CD38 were performed and ROC curve analysis was used to confirm the accuracy of these markers in predicting c-myc translocation. RESULTS: Of 54 cases, there were 33 males and 21 females with median age of 56 years. Histological types of lymphomas included 49 cases of DLBCL (11 cases of germinal central B cell like and 38 cases of activated B cell like by Hans classification), 4 cases of DLBCL/BL and 1 case of BL. Eleven of 54 patients died within 97 months, with median survival of 42 months. Histologically, full-thickness infiltration of the gastrointestinal tract by large atypical cells with evident phagocytosis of karyorrhexis by macrophages ("starry sky") were seen in 18/54 cases. The lymphoma cells were positive for CD20 (54/54), CD79a (54/54), CD43 (4/54), CD5 (7/54), bcl-2 (26/54), Tcl-1 (17/54) and CD38 (15/54), but all negative for CD3 and CD30. The proliferative index by Ki-67 ranged from 40% to 100%. The univariate survival analysis indicated that B symptoms, general performance, high LDH, high IPI, distant metastasis, high clinical stage and tumors with over 90% of cells positive for c-myc were negative predictors for the patient's survival. In addition, cases of DLBCL positive for CD5 had an unfavorable prognosis. Cox regression analysis showed c-myc translocation, distant metastasis and high LDH were independent predictors for unfavorable prognosis. ROC curve revealed the percentage of c-myc positivity predicted the presence of c-myc gene translocation, with 75% as the optimal threshold. CONCLUSIONS: Aggressive B-cell lymphomas of the gastrointestinal tract with a prognosis influenced by variable clinicopathologic factors. DLBCL and DLBCL/BL may possess c-myc translocation and tend to be Burkitt-like or atypical Burkitt lymphoma. As independent prognostic indicator, c-myc expression may be used for selection of therapeutic regimens and prognostication. High percentage of tumor cells with c-myc positivity may be used to predict the presence of c-myc gene translocation.

[Primary testicular yolk sac tumor: clinicopathological study of 8 cases].

OBJECTIVE: To investigate the clinicopathological characteristics, diagnosis and treatment of primary testicular yolk sac tumor (YST). METHODS: We studied 8 cases of primary testicular YST by microscopy and immunohistochemistry. RESULTS: The 8 cases of primary testicular YST, including 2 consultation cases, were confirmed from 1998 to 2013, accounting for 10.7% (8/75) of all the testicular germ cell tumors diagnosed in our hospital. The patients ranged in age from 7 to 43 years, 23.9 years on average. The main clinical manifestation of the patients was painless unilateral testis swelling. Microscopically, reticular tissues, schiller-duvaI (S-D) bodies, and eosin-stain transparent bodies were seen in the tumors. One of the cases was confirmed to be simple YST, while the other 7 mixed YST. AFP was a characteristic immunophenotype marker of the tumors. CONCLUSION: Primary testicular YST is a rare malignancyr with poor prognosis. Its diagnosis depends on preoperative AFP test and postoperative pathology. Comprehensive treatment, including orchiectomy, chemotherapy, and radiotherapy, can prolong the survival of the patients.

[Primary neuroendocrine tumor of the testis: clinicopathological study of 7 cases].

OBJECTIVE: To investigate the clinicopathologic characteristics, diagnosis, differential diagnosis and treatment of primary neuroendocrine tumor (NET) of the testis. METHODS: Using light microscopy and immunohistochemistry, we studied 7 cases of primary NET of the testis, reviewed relevant literature, and analyzed the clinical manifestations, histomorphologic and immunohistochemical characteristics, treatment and prognosis of the tumor. RESULTS: The 7 male patients, at the mean age of 40.6 years, all presented with testicular painless masses, none accompanied with carcinoid syndrome. Histologically, the uniform tumor cells were arranged in trabecular, island, solid and/or flake structures and locally in a tubulo glandular pattern, round and polygonal in shape, with a small amount of lipid vacuoles in the eosinophilic cytoplasm. The cells had round nuclei with fine chromatin and rarely identified mitosis. Immunohistochemical staining showed that the tumor cells were positive for Syn, CgA, NSE and CK, with a Ki-67 positive rate of < 2%. CONCLUSION: Primary NET of the testis is a rare and low-grade malignancy. Early diagnosis and surgical resection are essential for good prognosis. Immunohistochemistry helps its diagnosis and differential diagnosis from other metastatic neuroendocrine carcinoma, teratomas with carcinoid, seminoma, and Sertoli cell tumor.

Cathepsin K expression in a wide spectrum of perivascular epithelioid cell neoplasms (PEComas): a clinicopathological study emphasizing extrarenal PEComas.

AIMS: Recent studies have demonstrated that cathepsin K seems to be a powerful marker in identifying renal perivascular epithelioid cell neoplasms (PEComas). However, the expression in extrarenal PEComas has not been well characterized due to their rare incidence. Our aim was to investigate the expression of cathepsin K in a wide spectrum of extrarenal PEComas and evaluate its potential diagnostic usefulness in comparison with other commonly used markers. METHODS AND RESULTS: Twenty-three cases of PEComa (liver, n = 9; lung, n = 1; broad ligament of uterus, n = 1; vertex subcutaneous soft tissue, n = 1; abdominal wall, n = 1; and kidney, n = 10) were selected for study. All displayed a high percentage of cells with moderately to strongly positive reactions for cathepsin K (mean 91%; range 80-100%). HMB45, Melan-A and smooth muscle actin (SMA) were expressed in 78, 87 and 87% of cases, respectively, with various percentages of positive cells (mean, 34, 40 and 38%; range 0-80, 0-90 and 0-90%). Transcription factor E3 (TFE3) was expressed strongly in only three cases; none exhibited evidence of TFE3 gene fusion or amplification. CONCLUSIONS: Cathepsin K appears to be more powerful than other commonly used markers in diagnosing a wide spectrum of PEComas and distinguishing them from the majority of human cancers.

[Fibrosarcomatous dermatofibrosarcoma protuberans: a clinicopathological analysis of 12 cases].

OBJECTIVE: To investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP). METHODS: The clinical history, histopathological features and immunohistochemical characteristics were analyzed in twelve cases of FS-DFSP from January 1997 to February 2011, and related literature were reviewed. RESULTS: Age of the patients (2 females, 10 males) at diagnosis ranged from 41 to 70 years (mean 53 years). Among the 12 cases of FS-DFSP, 9 cases aroused in recurrent ordinary DFSP. Histologically, FS areas in FS-DFSP were characterized by a fascicular and highly cellular histology, frequently showing a characteristic herringbone pattern. FS-DFSP showed diminishment of CD34 staining in FS areas. The labeling index of Ki-67 was much higher in the FS areas (10%-40%) than that in the conventional DFSP areas (2%-5%). All the patients were treated by operation with local excision or wide excision. Postoperative radiotherapy and chemotherapy was administered in two cases respectively. Follow-up information in 9 of 12 patients (9 to 86 months) revealed local recurrence in 6 patients. Distant metastases were seen in two patients. One patient was died in the follow up period. CONCLUSIONS: FS-DFSP is a rare and unique subtype of DFSP and is associated with significant elevated risk of both local and distance metastasis, usually followed by poor outcome. Compared to ordinary DFSP as a borderline neoplasm, FS-DFSP should be considered as a malignant tumor.