CHAPTER X CHAPTER 4 - Cancer et environnement

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Fig. 4.24 Seminoma. Vascular invasion. vary in size from lymphocyte-like to giant cells of about 100 μm in diameter, with the bulk of the tumour composed of cells of intermediate size. ICD-O code 9063/3 Epidemiology Spermatocytic seminoma is rare, its frequency varying from 1.2 to 4.5 percent {347,1195,2565}. There is no difference in race predilection from other germ cell tumours. In a series of 79 cases {347} none of the patients had a history of cryptorchidism. Clinical features Most tumours occur in the older male with an average age of 52 years but it can also be encountered in patients in their third decade of life. Spermatocytic seminoma occurs only in the testis, unlike other germ cell tumours, which may be seen in the ovary and elsewhere. Most tumours are unilateral. Bilateral tumours are more often metachronous {220,347,2565}. Generally symptoms consist of painless swelling of variable duration {347}. Serum tumour markers are negative. Macroscopy The size ranges from 2 to 20 cm with an average of 7 cm {347}. The tumours are often soft, well circumscribed with bulging mucoid cut surfaces. They have been described as lobulated, cystic, haemorrhagic and even necrotic. Extension into paratesticular tissue has been rarely reported {2349}. Histopathology The tumour cells are noncohesive and are supported by a scant or oedematous stroma. The oedema may cause a “pseudoglandular” pattern. Collagen bands may enclose tumour compartments. Lymphocytic infiltration and granulomatous stromal reaction are only rarely seen. The tumour consists typically of 3 basic cell types {347,1195,1644, 1800,1805,2229,2349}. The predominant cell type is round of varying size with variable amounts of eosinophilic cytoplasm. Glycogen is not demonstrable. The round nucleus often has a lacy chromatin distribution with a filamentous or spireme pattern similar to that seen in spermatocytes. The second type is a small cell with dark staining nuclei and scant eosinophilic cytoplasm. The third cell type is a mono-, rarely multinucleated giant cell with round, oval or indented nuclei. These often have the typical spireme like chromatin distribution. Sometimes, the cells are relatively monotonous with prominent nucleoli Fig. 4.25 Spermatocytic seminoma. Note the mucoid appearence. although wider sampling reveals characteristic areas {55}. Mitoses, including abnormal forms are frequent. There may be vascular, tunical and epididymal invasion. The adjacent seminiferous tubules often show intratubular growth. The malignant germ cells (IGCNU) in adjacent tubules typically associated with other germ cell tumours are not present. Immunoprofile Many of the markers useful in other types of germ cell tumour are generally negative in spermatocytic seminoma. VASA is diffusely reactive {2929} PLAP has been observed in isolated or small groups of tumour cells {346,347,582}. Cytokeratin 18 has been demonstrated in a dot-like pattern {527,784}. NY-ESO-1, a cancer specific antigen, was found in 8 of 16 spermatocytic seminomas but not in other germ cell tumours {2299}. AFP, hCG, CEA, actin, desmin, LCA, CD30 are not demonstrable. CD117 (c-kit) has been reported to be positive {2299}, but others had negative results. Germ cell Fig. 4.26 Spermatocytic seminoma devoid of stroma and very edematous. 234 Tumours of the testis and paratesticular tissue
A B Fig. 4.27 Spermatocytic seminoma. A Note the three different cell types of spermatocytic seminoma. B Intratubular spread of spermatocytic seminoma. maturation stage specific markers, including SCP1 (synaptonemal complex protein 1), SSX (synovial sarcoma on X chromosome) and XPA (xeroderma pigmentosum type A1), have been demonstrated {2512}. Ultrastructure The cell membranes lack folds and indentations. There are intercellular bridges like those between primary spermatocytes {2226}. Gap junctions and macula adherens type junctions can be observed. The chromatin is either homogeneously dispersed or has dense condensations and nucleoli have net-like nucleolonema {2299}. Differential diagnosis Spermatocytic seminoma, when misinterpreted, is most frequently classified as typical seminoma or lymphoma. Seminoma, however, usually has a fibrous stroma, a lymphocytic and/or granulomatous stromal reaction and cells with abundant glycogen, PLAP positivity, and IGCNU component. Lymphoma has a predominant interstitial growth pattern and lacks the spireme chromatin distribution. Genetics The DNA content of spermatocytic seminoma is different from that of seminoma, including diploid or near hyperdiploid values {582,1832,2234,2568}. Small cells have been reported to be diploid or near diploid by cytophotometry {2555}, the intermediate cells have intermediate values and the giant tumour cells up to 42C. Haploid cells have not been reported {1385,2568}. These data are in keeping with the finding that spermatocytic seminoma cells show characteristics of cells undergoing meiosis, a feature that is diagnostically helpful {2512}. CGH and karyotyping show mostly numerical chromosomal aberrations. The gain of chromosome 9 in all spermatocytic seminomas appears to be a nonrandom chromosome imbalance {2234}. The presence of common chromosomal imbalances in a bilateral spermatocytic seminoma and immunohistochemical characteristics {2512} suggests that the initiating event may occur during intra-uterine development, before the germ cells populate the gonadal ridges. This might explain the relatively frequent occurrence of bilateral spermatocytic seminoma (5% of the cases). No gene or genes involved in the pathogenesis of spermatocytic seminomas have been identified yet, although puf-8 recently identified in C. elegans might be an interesting candidate {2524}. Prognosis Only one documented case of metastatic pure spermatocytic seminoma has been reported {1646}. Spermatocytic seminoma with sarcoma Definition A spermatocytic seminoma associated with an undifferentiated or, less frequently, with a differentiated sarcoma. Clinical features Approximately a dozen cases of this tumour have been reported. The age range is 34-68 years. There is no familial association, and no etiologic agents have been identified. The typical patient has a slowly growing mass that suddenly enlarges within months of diagnosis. Fifty percent of patients have metastases at diagnosis. Levels of serum alpha-fetoprotein and human chorionic gonadotropin are normal. Macroscopy Typically the tumour is a large (up to 25 cm), bulging mass with variegated cut surface exhibiting areas of induration, necrosis, and focal myxoid change. Histopathology The spermatocytic seminoma component frequently has foci of marked pleomorphism {647}, and is histologically contiguous with the sarcoma component. The sarcoma can exhibit various patterns - rhabdomyosarcoma, spindle cell sarcoma, and chondrosarcoma {347,783, 1649,1800,2646}. Differential diagnosis The primary differential diagnosis is sarcomatous transformation of a testicular germ cell tumour {2665}. Absence of teratoma and recognition of the spermatocytic seminoma excludes this possibility. The differential diagnosis of a tumour where only the sarcoma component is sampled includes primary testicular sarcoma {408,2786,2950}, paratesticular sarcoma, and metastatic sarcoma or sarcomatoid carcinoma {510,753,769, 2146}. Tumour spread and prognosis The sarcomatous component metastasizes widely. Most patients die of metastatic tumour, with a median sur- Germ cell tumours 235
Page 1 and 2: CHAPTER 4X Tumours of the of the Te
Page 3 and 4: TNM classification of germ cell tum
Page 5 and 6: Germ cell tumours P.J. Woodward A.
Page 7 and 8: senting symptom: back or abdominal
Page 9 and 10: A B Fig. 4.03 Germ cell tumours gen
Page 11 and 12: process at all {1524}. In addition,
Page 13 and 14: A B Fig. 4.12 Comparison of morphol
Page 15 and 16: small tumour insufficient to produc
Page 17: A B C Fig. 4.22 Seminoma. A Pseudog
Page 21 and 22: Differential diagnoses Differential
Page 23 and 24: Histologic patterns Microcystic or
Page 25 and 26: A Fig. 4.40 Yolk sac tumour. A Pleo
Page 27 and 28: Teratoma Teratomas are generally we
Page 29 and 30: A B C Fig. 4.49 Teratoma. A Teratom
Page 31 and 32: A B Fig. 4.52 Teratoma with somatic
Page 33 and 34: A B Fig. 4.59 Mixed germ cell tumou
Page 35 and 36: A B C Fig. 4.64 Leydig cell tumour.
Page 37 and 38: A Fig. 4.69 Androgen insensitivity
Page 39 and 40: tinguished from Sertoli cell nodule
Page 41 and 42: ound and have spherical, regularly
Page 43 and 44: Tumours containing both germ cell a
Page 45 and 46: Miscellaneous tumours of the testis
Page 47 and 48: Lymphoma and plasmacytoma of the te
Page 49 and 50: Tumours of collecting ducts and ret
Page 51 and 52: Tumours of paratesticular structure
Page 53 and 54: Epidemiology Nodular mesothelial hy
Page 55 and 56: A B Fig. 4.104 Papillary cystadenom
Page 57 and 58: Fig. 4.109 Desmoplastic small round
Page 59 and 60: Fig. 4.115 Leiomyosarcoma. Coronal,
Page 61 and 62: Secondary tumours C.J. Davis Defini

CHAPTER X CHAPTER 4 - Cancer et environnement

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