Small cell carcinoma of the bladder: a contemporary clinicopathological study of 51 cases.

AIMS: We present 51 cases of primary small cell carcinoma of the bladder in a clinicopathological study with emphasis on features that aid in the initial recognition and diagnosis of small cell carcinoma of the bladder. METHODS AND RESULTS: The patients were 40 men and 11 women between the ages of 39 and 87 years (mean age 67 years). Clinical data were available in 41 cases. The most common symptomatology was haematuria in 63% of the patients while dysuria was present in 12%. Thirty-eight patients were caucasians; seven patients were Hispanics; two patients were Asian; one patient was African-American; in the three additional patients no racial information was obtained. Biopsy material was obtained in all of the patients. Cystectomy was performed in 20 patients. At diagnosis, clinical stage was as follows: stage I in two (5%), stage II in 18 (44%), stage III in 10 (24%), and stage IV in 11 (27%). Histologically, urothelial carcinoma was present in 70% of the cases, adenocarcinoma in 8%, and squamous cell carcinoma in 10% of the cases. Small cell carcinoma was the only histology present in only 12% of the cases studied. Immunohistochemical studies using chromogranin, synaptophysin and chromogranin were positive in 30-70% of the cases. CONCLUSIONS: The present study highlights the unusual phenomenon of pure small cell carcinoma of the bladder and its association with other non-small cell carcinomas in that anatomical location. In addition, the study highlights the different modalities employed to treat patients in whom there is a component of small cell carcinoma of the bladder.

Primary signet-ring cell carcinoma of lung: immunohistochemical study and comparison with non-pulmonary signet-ring cell carcinomas.

Signet-ring cell carcinoma (SRCC) of lung is a rare variant of pulmonary adenocarcinoma. In view of this rarity, the question of whether an SRCC is primary pulmonary or metastatic arises frequently because the majority of SRCCs seen in lung are metastatic tumors having arisen in stomach, colon, or breast. On routine histologic examination it is difficult to distinguish between pulmonary SRCC from SRCC metastasizing from other organs. Thyroid transcription factor-1 (TTF-1) is a homeodomain-containing transcription factor that is almost exclusively expressed in thyroid and pulmonary epithelial cells. TTF-1 expression has been demonstrated in various neoplasms of lung; however, the expression of TTF-1 in SRCCs has not been investigated so far. In the present study, using an immunoperoxidase staining procedure on paraffin sections, we investigated the expression of TTF-1, cytokeratin 7, cytokeratin 20, and villin (a specific marker expressed in tumors of the digestive tract, renal proximal tubules, and hepatic bile ducts) in 32 SRCCs from various organs (17 lung, 5 breast, 5 stomach, and 5 colon). Fourteen (82.4%) of 17 pulmonary SRCCs exhibited TTF-1 positivity, whereas none of the SRCCs of other organs were positive for TTF-1. A cytokeratin profile (CK7+/CK20-) was identified in 94.1% of pulmonary SRCC, and although it differed from the profile exhibited in colonic SRCCs (CK7-/CK20+), a similar profile was seen in breast SRCCs and some SRCCs arising in the stomach. Villin was identified in 29.4% of pulmonary SRCCs and 20% (one case) arising in the breast. Although the pattern of villin immunostaining exhibited by nondigestive tract SRCCs (cytoplasmic) differed from those of digestive tract SRCCs (membranous), distinguishing between the two groups based on their pattern of immunostaining alone would be difficult. The results of this study indicate that TTF-1 is expressed in a high percentage of pulmonary SRCCs and is very specific and that TTF-1 would be extremely valuable in distinguishing pulmonary SRCCs from those arising in other organs.

Case report: Calcifying fibrous pseudotumour of the adrenal gland.

Calcifying fibrous pseudotumour is a distinct pathological entity usually occurring in the soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum. This report describes the hitherto unreported occurrence of this tumour of the adrenal gland in a 10-year-old girl whose imaging findings closely resembled a neuroblastoma. This entity is a potential pitfall in diagnosing adrenal neuroblastoma.

Adult neuroblastoma of the retroperitoneum and abdomen: clinicopathologic distinction from primitive neuroectodermal tumor.

Adult neuroblastoma (ANB) is a rare and poorly recognized entity among a histologically defined group of small, round-cell tumors arising in the retroperitoneum and abdomen. Eight cases of ANB were compared with seven cases of primitive neuroectodermal tumor (PNET) in these locations to identify clinicopathologic features that could be used to distinguish between the two lesions. The ANB study group included four men and four women 22-74 years of age (mean 38 years). Five patients with ANB presented with inflammatory symptoms or elevated levels of catecholamines and their metabolites. Five of the ANB tumors were classified as undifferentiated and three as poorly differentiated with a background of neuropil. These cases often showed immunoreactivity for multiple neural markers such as CD56, chromogranin A, synaptophysin, neurofilament, and neuron-specific enolase, but were negative for CD99, cytokeratins, desmin, myogenin, smooth muscle actin, muscle-specific actin, CD34, S-100 protein, and CD45. In contrast, all of the PNETs were positive for CD99, and four (57%) were also positive for cytokeratins. Two cases of ANB of the undifferentiated subtype had ultrastructural features characteristic of neuroblastoma and lacked a chimeric transcript (EWS-FLI1or ERG), which is specific for PNET. All five patients with the undifferentiated subtype of ANB and six of the seven patients with PNET died of their disease within 3 years of discovery of the lesion. Our results show that ANB, although rare, should be considered in the differential diagnosis of patients with small, round-cell tumors in the retroperitoneum and abdomen. Appropriate immunohistochemical studies and laboratory examination enable pathologists to distinguish ANB from other differential diagnoses, especially PNET.

Tumor stage, vascular invasion and the percentage of poorly differentiated cancer: independent prognosticators for inguinal lymph node metastasis in penile squamous cancer.

PURPOSE: We determine if histopathological factors of the primary penile tumor can stratify the risk of the development of inguinal lymph node metastases. MATERIALS AND METHODS: Clinical records of 48 consecutive patients with squamous cell carcinoma of the penis who underwent resection of the primary lesion and either inguinal lymph node dissection or were observed for signs of recurrence (median followup 59 months) were reviewed. Parameters examined included pathological tumor stage, quantified depth of invasion and tumor thickness, histological and nuclear grade, percentage of poorly differentiated cancer in the primary tumor, number of mitoses and presence or absence of vascular invasion. Variables were compared in 18 lymph node positive and 30 lymph node negative cases. RESULTS: Pathological tumor stage, vascular invasion and presence of greater than 50% poorly differentiated cancer were the strongest predictors of nodal metastasis on univariate and multivariate regression analyses. None of 15 pT1 tumors exhibited vascular invasion or lymph node metastases. Of 33 patients with pT2 or greater tumors 21 (64%) had vascular invasion and 18 (55%) had metastases. Only 4 of 25 patients (15%) with 50% or less poorly differentiated cancer in the penile tumor had metastases compared with 14 of 23 patients (61%) with greater than 50% poorly differentiated cancer (p = 0.001). No other variables tested were significantly different among the patient cohorts. CONCLUSIONS: Pathological stage of the penile tumor, vascular invasion and greater than 50% poorly differentiated cancer were independent prognostic factors for inguinal lymph node metastasis. Prophylactic lymphadenectomy in compliant patients with pT1 lesions without vascular invasion and 50% or less poorly differentiated cancer does not appear warranted.

Ciliated epithelia in the urethra: case report and literature review.

The presence of ciliated epithelial cells in the urethra has not been well recognized. Only two reports in the literature, both of which used scanning microscopy studies, have described this phenomenon. In this report, we illustrate the presence of scattered, ciliated epithelial cells in penile urethral biopsy specimens from a 38-year-old man with a history of bladder calculi and hematuria, by both light and transmission electron microscopy studies. The cilia in the urethra showed typical light microscopic and ultrastructural features of those seen in other organs. These ciliated cells are present in association with urothelial papilloma, condyloma acuminatum and acute inflammation of the urethra. These findings suggest that ciliated cells in the penile 0 urethra might be a consequence of metaplastic change of the urothelium, secondary to local stimulation or irritation.

Overexpression of cathepsin B and urokinase plasminogen activator is associated with increased risk of recurrence and metastasis in patients with chondrosarcoma.

BACKGROUND: Deregulation of the cellular protease network has been shown to be responsible for aggressive clinical behavior in several common human malignancies. In the current study, the authors evaluated the expression patterns of proteases in patients with chondrosarcoma of bone and correlated these patterns with clinical outcome. METHODS: The expression levels of urokinase plasminogen activator; matrix metalloproteinase types-1, -2, and -9; and cathepsins B and L were determined immunohistochemically in 114 cases of chondrosarcomas of bone and were correlated with their clinicopathologic parameters as well as with long term follow-up data. RESULTS: Overexpression of cathepsin B was associated with a high rate of local recurrence (P = 0.006) and a decreased recurrence free survival (P = 0.005). Overexpression of urokinase plasminogen activator was associated with an increased rate of metastasis (P = 0. 013), a decreased metastasis free survival (P = 0.016), and a decreased 5-year overall survival rate (P = 0.048). The univariate Cox model showed that tumor extension into soft tissue, high histologic grade, and overexpression of cathepsin B were predictors of adverse outcome. Multivariate analysis showed only overexpression of cathepsin B and tumor extension into soft tissue to be independent predictors of local recurrence. CONCLUSIONS: Overexpression of cathepsin B and urokinase plasminogen activator can be used to identify those patients with chondrosarcoma of bone who have an increased risk of local recurrence and distant metastases.

Gains and losses of DNA sequences in childhood brain tumors analyzed by comparative genomic hybridization.

Primary central nervous system neoplasms are the most common solid tumors in children. Genetic changes underlying childhood brain-tumor development and progression are incompletely characterized. To get an overview of the genetic events leading to the development of brain tumors and to identify chromosomal regions that may contain genes important in brain-tumor progression, we employed a comparative genomic hybridization technique. Twenty-four pediatric primary brain tumors were analyzed in this study. DNA copy number changes were observed in most of the samples (79%), and almost all chromosomes were involved. The total number of genetic aberrations (copy-number gains and losses per tumor) was significantly higher in the cerebellar primitive neuroectodermal tumor subgroup than in the gliomas. The high-grade tumors had more DNA changes than did the low-grade tumors. The most often gained chromosomes were: 6q (45%), 4q (34.5%), and chromosome 1 (24% of the cases). The minimal common regions involved in DNA gains were narrowed down to 6q14-16 and 4q26-28. Losses of a specific chromosome (partly or as a whole) occurred on average in 7% of the cases. Chromosomal regions that were most often lost included chromosome 1 (17%), chromosome 16 (17%), and chromosome 2 (14%). These findings suggest that genes localized to these minimal common chromosomal regions play a role in the tumorogenesis of childhood brain tumors. Our results indicate: (1) a great degree of genomic imbalance in these tumors; (2) that a high number of aberrations correlate with aggressive tumor biology; (3) and that nonrandom genetic changes may be associated with particular tumor types.

Fine-needle aspiration cytology of a case of oncocytic adrenocortical carcinoma.

We report on the results of fine-needle aspiration cytology of a case of oncocytic adrenocortical carcinoma in a 39-yr-old man. The tumor invaded the inferior vena cava and extended up to the right atrium. Aspirate smears were very cellular and showed a monomorphic population of large polyhedral cells with abundant granular cytoplasm, predominantly distributed singly. Mitotic activity was inconspicuous, and there was no necrosis. Immunohistochemically, the tumor cells were positive for vimentin, cytokeratin, and p53, and negative for synaptophysin, chromogranin, inhibin, and S-100. Ultrastructurally, the cytoplasm of the tumor cells was packed with mitochondria. The patient underwent left radical nephrectomy as well as a combined cardiopulmonary bypass, with atriotomy and resection of the tumor from the right atrium and inferior vena cava. Three months of postoperative follow-up were uneventful.

Pagetoid bowen disease: a report of 2 cases that express cytokeratin 7.

Bowen disease is a variant of squamous cell carcinoma in situ. In some cases a pagetoid growth pattern can be observed with cytologically atypical clear cells arranged singly and in nests. The differential diagnosis of pagetoid Bowen disease includes primarily Paget disease and malignant melanoma in situ, as well as other less common entities. Two cases of pagetoid Bowen disease are described, one in a 65-year-old man with a thigh lesion and the other in a 25-year-old man with a lesion in the penile/scrotal region. Neither patient had clinical evidence of an internal malignant neoplasm. In both cases, the neoplastic cells were positive for cytokeratin (CK) 7 and CK 19 and were negative for CK 18, CK 20, carcinoembryonic antigen, GCDFP-15, c-erbB2, S100, and HMB-45. In aggregate, these findings support the diagnosis of pagetoid Bowen disease. Previously, others have shown that CK 7 is an almost invariable marker of Paget disease. Thus, we report these two cases to illustrate that CK 7 can be expressed by pagetoid Bowen disease and should not be a cause of confusion in the differential diagnosis.

Benign tumors and tumor-like lesions of the adult kidney. Part II: Benign mesenchymal and mixed neoplasms, and tumor-like lesions.

In this review article the benign tumors and tumor-like lesions of the adult kidney are discussed. The incidence of benign renal tumors is low, especially when compared to renal cell carcinomas, as most are detected incidentally or at autopsy. Some of these tumors, as their names imply, are unique to the kidney, e.g., renal adenoma, metanephric adenoma, renal oncocytoma, nephrogenic adenofibroma, mesoblastic nephroma, capsuloma, juxtaglomerular cell tumor, renomedullary interstitial cell tumor (medullary fibroma), cystic nephroma, cystic partially differentiated nephroblastoma, and cystic hamartoma of the renal pelvis, while others, such as angiomyolipoma, leiomyoma, hemangioma, lipoma, etc., are not unique to the kidney and show similar morphologic features in the other sites they affect. Of the tumor-like lesions, xanthogranulomatous pyelonephritis, malakoplakia, and renal cysts are the most common. The other entities, such as fibroepithelial polyp, are rare, most having been the topic of case reports. In Part I of this paper the benign epithelial tumors of the kidney were previously discussed. This paper (Part II) is devoted to the benign mesenchymal tumors, mixed mesenchymal and epithelial tumors, and the tumor-like lesions.

Extrapleural solitary fibrous tumor: a clinicopathologic study of 24 cases.

Solitary fibrous tumors (SFTs), rare in extrapleural sites, can present difficulties in diagnosis at these locations. From the files of the M. D. Anderson Cancer Center, we accessed 24 cases of extrapleural SFT (14 females, 10 males, ages 19 to 85 yr) obtained for clinical, histologic, immunohistochemical, and follow-up findings. Tumor locations included the head and neck (n = 12), the abdomen (n = 10), and the extremities (n = 2). Tumors were 2 to 25 cm in greatest dimension (mean, 8.2 cm) and were well circumscribed or encapsulated. Histologic features were typical of their pleural counterparts, e.g., bland spindle cells with some hypercellular areas and myxoid to hyalinized backgrounds. A hemangiopericytic vascular pattern was present in 19 cases and prominent in 11. Mitotic activity ranged from 0 to 4 counts per 10 high-power fields. Necrosis was seen in two cases. Focally increased cellularity was seen in seven cases and margins were positive in another seven cases. Spindle cells were positive for vimentin (19 of 19) and CD34 (18 of 20), and negative for cytokeratin (0 of 19). Fibroblastic differentiation was present in the three cases studied by electron microscopy. Flow cytometry in three cases revealed diploid cell populations. Follow-up for 19 cases (9 to 99 mo) revealed no evidence of metastasis, although one patient had residual tumor after an incomplete excision, and one patient died of unrelated causes. Histologic findings such as mitotic counts, necrosis, cellularity, and marginal status were not associated with outcome. We conclude that extrapleural SFTs are clinically and histologically similar to their pleural counterparts. Although the length of clinical follow-up was short (mean follow-up, 41.4 mo), recognizing these lesions is important because they typically follow an indolent clinical course if completely excised. Although CD34 is nonspecific, it is usually positive in SFTs and may aid in their diagnosis.

Dermatofibrosarcoma protuberans of the vulva: clinicopathologic and immunohistochemical analysis of four cases, one with fibrosarcomatous change, and review of the literature.

Only 18 cases of dermatofibrosarcoma protuberans (DFSP) of the vulva have been reported. This article describes the clinicopathologic and immunohistochemical findings of four additional vulvar DFSPs. The median patient age was 54 years (range, 36 to 69 years) and the most common clinical presentation was a slowly growing vulvar mass. The tumors occurred in the left paraclitoral area (1 case), right labium majus (1 case), left labium majus (1 case), and mons pubis (1 case). The tumor size, known in three cases, ranged from 1.2 to 5.0 cm in greatest dimension. Microscopically, the tumors were composed of bland spindle cells with oval or wavy nuclei arranged in a cartwheel pattern; in one case, fibrosarcomatous areas were apparent. CD34 immunostaining was positive in the three cases in which it was performed; positive staining was also seen in the area of fibrosarcoma. Estrogen and progesterone receptor immunostains were negative in three cases. Of the three patients who underwent surgery, one had tumor recurrences every 2 to 4 years for 18 to 20 years but has remained free of tumor in the 7 years since the last excision, one was free of tumor for 12 years before dying of an unrelated cause, and the other has been free of disease for 6 months. The fourth patient refused treatment and has persistent disease in the vulva. This study shows that the behavior of DFSP in the vulva is similar to its behavior in its more common locations.

Colonic adenocarcinoma metastatic to the thyroid gland: a case report with immunohistochemical investigation.

Clinically evident metastases of carcinomas to the thyroid gland are rare, particularly from a colorectal primary tumor. We present a case of colonic adenocarcinoma metastatic to the thyroid gland with histopathologic and immunohistochemical findings. A 68-year-old woman with a history of Dukes' stage B colon carcinoma presented a mass in the thyroid gland. The tumor was confirmed to be metastatic adenocarcinoma from the colon. The immunohistochemical findings demonstrated positive staining for cytokeratin 20, low-molecular-weight cytokeratin, villin and carcinoembryonic antigen, but stains were negative for cytokeratin 7 and thyroglobulin.

Metastatic renal cell carcinoma to the bladder: a clinicopathologic and immunohistochemical study.

Although rare, renal cell carcinoma (RCC) can metastasize to the bladder. When this occurs, it might complicate diagnosis. Morphologically, RCC can be confused with transitional cell carcinomas (TCCs), especially those exhibiting clear cell features, and also with other bladder tumors, such as paragangliomas and metastatic melanomas. We report seven cases of RCC metastatic to the bladder that occurred in 6 men and 1 woman who were 35 to 69 years old. The most common presenting symptom was the reappearance of hematuria, which developed from 2 to 131 months (mean, 41.3 mo) after the removal of the primary RCC. In all of the patients, the metastatic RCC involved multiple organs; no case had an isolated metastasis to the bladder. The prognosis was poor, and five patients died of disease between 4 and 24 months (mean, 12.8 mo) after diagnosis of the metastasis to the bladder. The remaining two patients were lost to follow-up. All of the tumors were conventional clear or "granular" cell RCCs, with nuclear grades of 2 or 3. In five patients, metastases were confined to the lamina propria, but in two patients, tumors involved the muscularis propria as well. A comparative immunohistochemical study showed that metastatic RCCs were positive for CAM5.2, vimentin, and Leu-M1, and negative for cytokeratin 20, cytokeratin 7, 34betaE12, carcinoembryonic antigen, S-100 protein, HMB45, and chromogranin. Classic and clear cell TCCs were positive for all of the cytokeratins and carcinoembryonic antigen and negative for vimentin. Paragangliomas were positive for chromogranin and showed scattered positivity for the S-100 protein in the sustentacular cells. Metastatic melanomas were positive for S-100 protein and HMB45. The histologic appearance of RCC, particularly the delicate fibrovascular stroma with abundant sinusoidal vessels, is a feature that can be used to recognize the tumor. When there is difficulty diagnosing metastatic RCC, TCC, or other tumors in the bladder, the immunohistochemical findings can assist in the differential diagnosis.

Benign tumors and tumor-like lesions of the adult kidney. Part I: Benign renal epithelial neoplasms.

The spectrum of renal neoplasms has expanded in recent years. Although most of the work taking place in this field has concerned malignant neoplasms of the kidney, there have been significant improvements in our knowledge of benign renal tumors and tumor-like lesions, especially in renal cell adenoma, renal oncocytoma, and renal angiomyolipoma. Awareness and knowledge of these benign lesions is important because they are often included in the differential diagnoses of malignant tumors, with which they may be confused both clinically and pathologically. The authors review the topic of benign renal neoplasms and tumor-like lesions that occur in adults, emphasizing some of the newly described aspects of these lesions.

Protease expression in dedifferentiated parosteal osteosarcoma.

BACKGROUND: Parosteal osteosarcoma with dedifferentiation provides a useful model to study tumor progression from an indolent locally aggressive neoplasm to highly lethal metastasizing malignancy. Up-regulation of the proteolytic enzymes participating in stromal degradation is known to promote invasive growth and metastasis of several human and experimental tumors. METHODS: The expression patterns of urokinasase plasminogen activator (u-PA), its cell-surface receptor (u-PAR), and cathepsin B were analyzed by immunohistochemical techniques in 11 cases of parosteal osteosarcoma and in 4 cases of dedifferentiated parosteal osteosarcoma. RESULTS: Both enzymes and the receptor were coexpressed in most tumor cells of parosteal and dedifferentiated parosteal osteosarcoma. Their expression was strikingly enhanced in the dedifferentiated high-grade component of the tumors. Tumor cells involved in bone production (ie, those adjacent to tumor produced bone trabeculae) exhibited equally strong expression of u-PA, u-PAR, and cathepsin B, regardless of their histologic grade. Expression of u-PA, u-PAR, and cathepsin B was undetectable in the "normalized" cells embedded in the well-developed tumor bone trabeculae. CONCLUSION: These data indicate that u-PA and its interacting molecules, such as u-PAR and cathepsin B, may have some contributory effects on the metastatic potential of tumor cells in dedifferentiated parosteal osteosarcoma.

Altered p53 is associated with aggressive behavior of chondrosarcoma: a long term follow-up study.

BACKGROUND: p53 is a major tumor suppressor gene that has been implicated in the biology of a variety of human neoplasms, including some that affect the skeleton. Recent studies based on small numbers of cases have shown that overexpression or alteration of the p53 gene is frequently present in high grade, clinically aggressive chondrosarcomas of bone. In this study, the authors addressed the relation between overexpression and alteration of the p53 gene and the clinical aggressiveness of chondrosarcoma in a large series of patients for whom long term follow-up data were available. METHODS: The authors analyzed the expression and/or alteration of the p53 gene in 158 cases of chondrosarcoma of bone using immunohistochemistry, single-strand conformation polymorphism, and direct sequencing. They then related the findings to various clinicopathologic parameters and long term follow-up data. RESULTS: The presence of overexpression and/or structural alterations of the p53 gene was documented in 38.1% of chondrosarcomas of bone. A statistically significant correlation was observed between overexpression or alteration of the p53 gene and both the histologic grade of the tumor and the presence of metastasis. The probability of local recurrence free, metastasis free, and overall survival was significantly higher for patients with no overexpression or alteration of p53 than for patients with p53 overexpression or alteration. CONCLUSIONS: Overexpression or alteration of the p53 gene is an important predictor of aggressive clinical behavior in chondrosarcoma of bone.

Histologically pure seminoma with elevated alpha-fetoprotein: a clinicopathologic study of ten cases.

Seminomas account for 50% of testicular germ-cell tumors, and more than 90% of these are classic seminomas. When patients with a histologically pure testicular seminoma show an elevated level of serum á-fetoprotein (AFP), it is generally assumed that an undetected focus of yolk sac tumor (YST) is present and the patient is managed with a treatment regimen for non-seminomatous tumor. We studied 10 cases of histologically pure seminoma with elevated levels of serum AFP in an attempt to identify any distinctive clinical, histopathologic, or immunohistochemical features. The patients ranged in age from 27 to 48 years (mean, 31 years). Eight patients had primary tumors of the testis, and two presented with supraclavicular and ileal tumors. The clinical stage at presentation varied: four tumors were stage I, four were stage II, and two were stage III. Serum levels of AFP were elevated in all patients at ranges of 10.4 to 16 ng/ml (mean, 12.0 ng/ml). In all patients, the primary tumors and metastases when present exhibited classic seminoma histology without other germ-cell components. The tumor cells expressed keratin in seven cases. The pattern of keratin immunoreactivity ranged from focal staining in five cases to moderate staining in two cases. All cases were negative for AFP, and the nine cases in which staining for CD30 (Ki-1) was performed were also negative. All four patients with stage I tumors underwent the conventional therapy for pure seminoma, i.e., orchiectomy and subsequent radiation therapy. Five patients received treatment for non-seminomatous tumors, i.e., chemotherapy after orchiectomy. Extensive work-up failed to detect the primary tumor in one patient, and he was treated for a non-seminomatous tumor, undergoing chemotherapy and irradiation. All patients are alive and well, and none has developed evidence of YST at a mean follow-up of 6 years (range, 6 months to 10 years). However, one patient who presented with an ileal metastasis recently developed a second primary extragonadal mediastinal mixed germ-cell tumor with YST and embryonal carcinoma components and an elevated serum level of AFP (27,000 ng/ml) after a 10-year disease-free follow-up. This study strongly suggests that minor elevations (

Male adnexal tumour of probable Wolffian origin occurring in a seminal vesicle.

AIMS: Adnexal tumours of probable Wolffian origin are rare low-grade malignant neoplasms that have been previously described in the broad ligament, ovaries and retroperitoneum of females. All are characterized by small, bland epithelial cells growing in a diffuse, trabecular, or tubular pattern. The majority of the cases reported have pursued a benign clinical course. However, recurrences and distant metastases have been described. We present a case of a male adnexal tumour of probable Wolffian origin occurring in the left seminal vesicle of a 29-year-old man with 23 years of follow-up. RESULTS: The diagnosis is supported by immunohistochemical and electron microscopic findings: The tumour cells were immunoreactive for cytokeratin and vimentin while smooth muscle antigen and S100 protein were uniformly negative. By electron microscopy cells were arranged in an acinar pattern and surrounded flocculent, electron-dense material. Individual cells contained numerous dense bodies and free ribrosomes. The patient had recurrences at 14 and 23 years after initial diagnosis. CONCLUSION: This is the first report of this entity in a male. The literature on this unusual tumour is reviewed and the clinicopathological, immunohistochemical and ultrastructural features are described. The differential diagnosis of this seemingly indolent tumour is discussed with genitourinary tumours having a more aggressive clinical course.