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PubMed1997

[Management of stage I testicular nonseminomatous germ cell tumors with an embryonic carcinomatous component. 18 cases].

OBJECTIVE: To evaluate the prognosis and therapeutic modalities of stage I nonseminomatous germ cell tumours of the testis (NSGT) with an embryonic carcinomatous component (EC). MATERIAL AND METHODS: 18 patients with stage I nonseminomatous germ cell tumour of the testis with an embryonic carcinomatous component were treated between 1987 and 1995. EC represented more than 50% of the testicular tumour mass in 15 cases. This tumour contingent constituted the only potential prognostic factor in 4 cases, but vascular or lymphatic emboli (n = 3), tumour stage > pT1 (n = 5) or absence of endodermal sinus component (n = 9) were observed in 14 cases. The first 3 patients underwent retroperitoneal lymph node dissection and the following 15 patients were submitted to surveillance (n = 4) or chemotherapy (n = 11) according to the PVB [Cisplatin, Vinblastine, Bleomycin] (n = 7) or BOE [bleomycin, Etoposide, Cisplatin] (n = 4) protocols. RESULTS: With a follow-up of 10 to 110 months (mean: 46), the survival rate is 100% and the recurrence rate is 22%. None of the patients with a local stage exceeding pT1 relapsed after chemotherapy. 2 patients in whom the EC contingent represented less than 50% of the tumour mass and who were simply watched, did not relapse. 4 relapses, detected 3 to 14 months after orchidectomy (mean: 8.5), during surveillance (n = 2) or after chemotherapy (n = 2), required surgical resection or complementary chemotherapy. They occurred in patients in whom EC represented more than 50% of the testicular lesion. The tumour of initially conservatively managed patients did not contain an endodermal sinus component (n = 2) or presented vascular emboli (n = 1). The subjects treated by chemotherapy were characterized by the presence of emboli (n = 1) or the absence of endodermal sinus component (n = 1). The course after recurrence was favourable in 3 cases and the last patient is currently receiving chemotherapy. CONCLUSION: EC is an independent risk factor whose presence justifies proposal of complementary treatment by retroperitoneal lymph node dissection or chemotherapy, possibly limited to 2 courses of BOE. Surveillance can only be considered in the case of a minority of EC in the tumour, in the absence of any associated risk factors.

Adolescent↗
PubMed2002

Mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient.

We report a case of a mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient. Rhabdomyosarcoma is one of the most common childhood tumors; however, it rarely occurs in the elderly. In addition, to our knowledge, paratesticular rhabdomyosarcoma and adenomatoid tumor rarely form mixed tumors. Finally, we consider the pathogenesis of diploblastic tumors.

Aged↗
PubMed2001

Nonepithelial neoplasms of the urinary bladder.

Nonepithelial tumors are rare in the urinary bladder, but their exact classification is very important in the differential diagnosis between these tumors and epithelial lesions. In the new WHO classification and in the third series of the Armed Forces Institute of Pathology (AFIP) "Atlas of Tumor Pathology" on urinary bladder tumors, various mesenchymal tumors, mixed epithelial and mesenchymal tumors and myofibroblastic proliferations are summarized. In the following we will describe the histology, immunohistology, and cytogenetics of nonepithelial tumors and lesions.

Biomarkers, Tumor↗
PubMedDate not supplied

Mixed tumors, myoepitheliomas, and oncocytomas of the soft tissues are likely members of the same family: a clinicopathologic and ultrastructural study.

Four diagnostically unusual soft tissue tumors are presented. All lesions were of consistent size and long duration. Histologically, one lesion was analogous to mixed tumors of the usual sites (i.e., salivary glands), one lesion was totally spindled, and the two other lesions both had oncocytic appearances (epithelioid and spindle biphasic pattern in a case, purely epithelioid in the other). Immunohistochemically, the mixed tumor was positive for vimentin, cytokeratins, S-100 protein, and focally for EMA. The purely spindled tumor exhibited immunoreactivity for vimentin, actins, S-100 protein, EMA (focally), and GFAP. The oncocytic biphasic tumor was positive for mitochondrial antigen, vimentin, and actins. The purely epithelioid oncocytic neoplasm was immunoreactive only for mitochondrial antigen and vimentin. Ultrastructurally, in the epithelial-like portion of the first (mixed) tumor, peripheral arrays of contractile filaments were detected along with well-developed desmosomes. In the second (spindled) case, peripheral contractile filaments and attenuated desmosomes were also seen. In the third case, a huge number of mitochondria, some desmosomes, and actin-type microfilaments were found. In the fourth case, desmosomes and punctate subplasmalemmal densities, in addition to numerous mitochondria, were documented. In all cases an external basal lamina were present, which was discontinuous in the first three cases and almost continuous in the fourth. These tumors were respectively designated as mixed tumor, myoepithelioma of the classic type, myoepithelioma of oncocytic type with biphasic cell architecture, and true oncocytoma. So far, all tumors have followed benign clinical courses (median follow up: 12 months). Comparisons with similar tumors of other sites are drawn, and suggestions for considering all of them as members of the same myoepithelial-derived tumor family are given.

Adenoma, Oxyphilic↗
PubMed2000

Thyroid carcinomas with mixed follicular and C-cell differentiation patterns.

Divergent endocrine-neuroendocrine differentiation in thyroid carcinoma occurs in mixed medullary-follicular carcinomas (MMFC). Less than 40 cases of MMFC have been reported having highly heterogeneous patterns of growth. Classical medullary carcinoma areas may be intermingled with follicles or papillae or even oxyphilic and solid areas. Calcitonin and thyroglobulin are expressed in different cell populations. Presence of the latter suggests a potential usefulness of radioiodine treatment. The clinical behavior of MMFC does not differ from that of ordinary medullary carcinoma. The histogenesis of MMFC is controversial. The genetic analysis of the 2 neoplastic components showed that they are not derived from a common precursor, but rather display remarkable differences in the genetic profile (RET mutations and allelic losses). In addition, in some cases the follicular component was found to be oligo/polyclonal and therefore possibly hyperplastic rather than neoplastic. The follicular cells may have grown into the medullary carcinoma, after acquiring some molecular defect, being "hostage" of the true neoplastic (medullary) component.

Adenocarcinoma, Follicular↗
PubMed2000

Adult mesoblastic nephroma.

We report a case of asymptomatic mesoblastic nephroma in a 54-year-old woman. The tumor showed immunohistochemical reactions similar to developing nephrons. Electron microscopy showed immature tubules with numerous intracytoplasmic intermediate filaments. Recent studies support the concept of pathogenesis of the mesoblastic nephroma originating from collecting ducts. However, this case exhibited a complex pattern of antigenic expression not restricted to the collecting ducts, but including the glycoprotein CD24 and the neural cell adhesion molecule (NCAM). The following differential diagnoses will be discussed: benign mixed epithelial and stromal tumor, metanephric adenoma, and nephrogenic adenofibroma.

Adenofibroma↗
PubMed2007

Desmoplastic small round cell tumor of the pleura.

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with aggressive behavior. Usually it presents as a peritoneal mass, although other cases in various locations have been described. Since less than 10 cases of primary DSRCT in the pleura have been described, it is of interest to report a pediatric case arising from the pleura. The diagnosis was confirmed by molecular detection of the EWS/WT-1 fusion gene product. Multidisciplinary treatment with chemotherapy, radiotherapy, and surgical resection resulted in a progression-free survival time above the median survival, suggesting that this conventional approach could prove effective for this rare and very aggressive malignancy.

Biomarkers, Tumor↗
PubMed2000

Divergent neuroendocrine differentiation in prostatic carcinoma.

A rich variety of neuroendocrine cells are present in the normal prostate gland. Prostatic carcinoma may show divergent differentiation towards a neuroendocrine phenotype in the form of neuroendocrine small cell carcinoma or carcinoid-like tumors. Much more common is focal neuroendocrine differentiation in prostatic adenocarcinoma which may be pronounced in approximately 10% of adenocarcinomas. The prognostic significance of focal neuroendocrine differentiation in prostatic carcinoma is controversial but current evidence suggests an influence on prognosis related to hormone resistant tumors and/or a role in the conversion to a hormonal resistant phenotype. Chromogranin A appears to be the best overall tissue and serum marker of neuroendocrine differentiation. Chromogranin A serum levels may be useful in the assessment of the emergence of and/or progression of hormone resistant cancer.

APUD Cells↗
PubMed2002

A case of salivary gland-type mixed tumor of the lung differentiating toward type II alveolar epithelial cells in glandular components with a literature review.

Salivary gland-type mixed tumor primarily arising in the lung is extremely rare. We report here a case of this type of tumor that occurred in the periphery of S4 of the right middle lobe in a 74-year-old man. Light-microscopically, this lung tumor, 15x9mm in size, exhibited almost the same features as those of mixed tumor of the salivary gland intermingled with chondromyxoid stroma, glandular component, solid growth pattern of myoepithelial components and well-developed cartilage formation, exhibiting a sharp margin. Immunohistochemical study revealed that the glandular components in the tumor was positive for thyroid transcription factor-1, TTF-1, a marker of epithelial cells of the thyroid as well as the lung. Furthermore, surface lining cells of the glandular components and luminal contents were positive for surfactant apoprotein A, PE-10, used as a marker of type II alveolar epithelial cells. These findings clearly demonstrate for the first time that glandular epithelial cells in the present salivary gland-type mixed tumor exhibited differentiation toward type II alveolar epithelial cells.

Aged↗
PubMed2002

Complexities in ETS-domain transcription factor function and regulation: lessons from the TCF (ternary complex factor) subfamily. The Colworth Medal Lecture.

The ETS-domain transcription factor family can be divided into a series of subfamilies. Elk-1 represents the founding member of the ternary complex factor (TCF) subfamily. By focusing on the TCF subfamily, we can demonstrate the complexities that exist in the function and regulation of ETS-domain transcription factors. This article focuses on Elk-1 in detail and summarizes the functions of other TCFs. The key themes covered include the domain structure of the TCFs, the mechanisms of complex formation with serum response factor, regulation of TCFs by mitogen-activated protein kinase cascades, and transcriptional regulatory properties of the TCFs. Finally, the emerging role of the TCFs in vivo is discussed. A picture is developing indicating that, while these proteins exhibit significant sequence and functional conservation, key differences in their structure and regulation are being identified which may relate to unique functions of these proteins in vivo.

Amino Acid Sequence↗
PubMed2004

Expression of bone morphogenetic protein-6 and bone morphogenetic protein receptors in myoepithelial cells of canine mammary gland tumors.

To study the ectopic chondrogenesis in canine mammary mixed tumors, the expression of bone morphogenetic protein-6 (BMP-6) and specific BMP receptors (BMPRs), BMPR-IA, BMPR-IB, and BMPR-II, was examined using immunohistochemical and immunoblot analysis in 39 canine mammary gland tumors. Immunohistochemically, BMP-6 and all three types of BMPRs were coexpressed in the myoepithelial cells and chondrocytes in six of eight benign mixed tumors. In complex adenomas, myoepithelial cells showed an expression pattern of BMP-6, BMPR-IA, and BMPR-II similar to those in benign mixed tumors, whereas immunoreactivity for BMPR-IB was very mild. The myoepithelial cells proliferating within the basement membrane showed more intense immunoreactivity for BMP-6 and all BMPRs as compared with those proliferating in the interstitial areas. Western blotting analysis revealed immunopositive bands at 40-45 kDa for BMP-6 in the samples from simple and complex adenomas and benign mixed tumors. The BMPR-IB-specific bands at 45 kDa were most detected in benign mixed tumors. Because among BMPRs, BMPR-IB is thought to be the major receptor for BMP-6 for primary chondrogenesis, these findings suggest that the expression of BMP and its receptors on the myoepithelial cells might play a role in the ectopic cartilage formation in canine mammary gland tumors, especially in benign mixed tumors.

Animals↗
PubMed2001

Mixed tumour of schwannoma and meningioma in a patient with neurofibromatosis-2 : a case report.

The co-existence of schwannoma and meningioma as a mixed intracranial tumour is uncommon and so far only eight cases have been published in the literature. Because of rarity, we report a unique case of mixed tumour having schwann cell and meningeal components, in a patient with neurofibromatosis type -2 (NF-2). The possible mechanisms for the occurrence of these mixed tumours are discussed.

Adult↗
PubMed2000

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.

Adenoma↗
PubMed1994

Pleuropulmonary blastoma is THE pulmonary blastoma of childhood.

Pulmonary blastoma was defined by Spencer and others as a neoplasm with histopathological features thought to be reminiscent of Wilms' tumor. Unlike the other embryonal-fetal neoplasms that typically occur in early childhood, the majority of pulmonary blastomas have been reported in adults. One explanation offered by Spencer for the delayed clinical presentation of pulmonary blastoma is the continued development of lung parenchyma well past the postnatal period. It has been proposed that the pulmonary blastoma is a variant of carcinosarcoma, which is seen almost exclusively in adults. The classic pulmonary blastoma is described as a neoplasm with a mixture of primitive tubular profiles, immature blastema, and a spindle cell stroma. More recently, some have included the well-differentiated adenocarcinoma of fetal type as a pure epithelial expression of pulmonary blastoma. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, either embryonal rhabdomyosarcoma or a complex, mixed blastematous and sarcomatous neoplasm. Some pulmonary blastomas in children, particularly those with only embryonal rhabdomyosarcoma, have presented as a peripheral multicystic lesion that has been interpreted as a developmental cyst, either cystic adenomatoid malformation or bronchogenic cyst. Other tumors have been described as intrathoracic and anatomically separate from the lung, like some extralobar sequestrations. We have proposed the designation pleuropulmonary blastoma for these pulmonary or extrapulmonary neoplasms of childhood. We propose that this neoplasm is the rightful pulmonary blastoma rather than the pulmonary blastoma of Spencer, which has been the long-time pretender to the title.

Adolescent↗
PubMed2005

Islets of meningioma in an acoustic schwannoma in a patient with neurofibromatosis-2: pathology and magnetic resonance imaging findings.

Mixed tumors of the cerebellopontine angle, composed of meningioma and schwannoma components, are extremely rare; so far, only 12 cases have been reported in the literature. They are thought to be exclusively associated with neurofibromatosis-2. We present a mixed tumor of schwannoma and meningioma in a patient with neurofibromatosis-2 and discuss the pathology and magnetic resonance imaging (MRI) findings in relation to the literature. Review of the literature shows that a typical MRI pattern has not been established for mixed tumors and it seems unlikely that a meningioma component can be differentiated within a schwannoma preoperatively.

Adolescent↗
PubMed2006

Benign mixed epithelial and stromal tumor of the kidney.

A 51-year-old, perimenopausal, female patient with 1-month history of right flank pain who was diagnosed with a renal mass and underwent nephron-sparing partial nephrectomy is presented. The renal mass was found to be a benign, biphasic tumor composed of an epithelial component, consisting of ducts of variable size scattered within a mesenchymal component, composed of spindle cells arranged in sheets and fascicles. No atypia, mitosis, or necrosis was found. The spindle component shows desmin, smooth muscle actin, and estrogen and progesterone receptor positivity immunohistochemically. The diagnosis of benign mixed epithelial and stromal tumor of the kidney is rendered. No recurrent disease has been detected during 2 years of follow up.

Epithelial Cells↗