CHAPTER X CHAPTER 4 - Cancer et environnement

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A B C Fig. 4.67 Malignant Leydig cell tumour. A Necrosis. B Pronounced nuclear and cellular pleomorphism. C Note abnormal mitosis in center. D Leydig cell tumour with spindle change. D Sertoli cell tumour Definition Sertoli cell tumour is a sex cord-stromal tumour of the testis composed of cells expressing to a varying degree features of fetal, prepubertal or adult Sertoli cells. ICD-O codes Sertoli cell tumour 8640/1 Sertoli cell tumour lipid rich variant 8641/0 Sclerosing Sertoli cell tumour Large cell calcifying Sertoli cell tumour 8642/1 Synonym Androblastoma. Epidemiology They account for less than 1% of all testicular tumours. Typically Sertoli cell tumours NOS occur in adults, and the mean age at the time of diagnosis, is around 45 years. Sertoli cell tumours NOS are only exceptionally found in men under the age of 20 years {2894}. Variant forms, and especially those that occur as parts of various syndromes, are more common in infants and children. The vast majority of Sertoli cell tumours are sporadic, but some tumours have been associated with genetic syndromes such as androgen insensitivity syndrome {2268}, Carney syndrome {2785}, and Peutz-Jeghers syndrome {2894}. Clinical features Signs and symptoms Patients harbouring Sertoli tumours of any type typically present with a slowly enlarging testicular mass {827}. Hormone related symptoms are not typical of Sertoli cell tumours {2894}. Sertoli cell tumours in boys with Peutz-Jeghers syndrome have signs of hyperestrinism {61,2907}. Imaging Sertoli cell tumours are generally hypoechoic. They can be variable echogenecity and cystic areas. The imaging characteristics are nonspecific and indistinguishable from germ cell tumours. An interesting subtype, which can often be distinguished, is the large cell calcifying Sertoli cell tumour. These Fig. 4.68 Sertoli cell tumour. Intratubular Sertoli cell tumour in a patient with Peutz-Jeghers syndrome. 252 Tumours of the testis and paratesticular tissue
A Fig. 4.69 Androgen insensitivity syndrome. A Sertoli - Leydig cell hamartomas in androgen insensitivity syndrome (AIS). B Sertoli cell hamartoma in center and nodular Leydig cell proliferation. masses can be multiple and bilateral and, as the name implies, are characterized by large areas of calcification which are readily seen by ultrasound {410,873}. Calcifications will app`ear as brightly echogenic foci, which block the transmission of sound (posterior acoustic Fig. 4.70 Sertoli cell tumour. Longitudinal ultrasound image of the testis shows a small well defined mass (arrow). It is slightly heterogeneous with small cysts (anechoic areas) within it. A B shadowing). This diagnosis is strongly suggested when calcified testicular masses are identified in the pediatric age group. Macroscopy Most tumours present as spherical or Fig. 4.71 Sertoli cell tumour of the testis. Fig. 4.72 A Large cell calcified Sertoli cell tumour shows bilateral, brightly echogenic masses with posterior acoustic shadowing (arrow). B Sertoli cell tumour. Large cell calcified Sertoli cell tumour. This case was malignant; note focus of yellow necrosis. B lobulated, well circumscribed masses, varying in size from 1 cm to more than 20 cm in diameter. The average size of tumours recorded in the largest series of 60 cases is 3.5 cm {2894}. On cross section the tumours appear tan-yellow or greyish white. Foci of haemorrhage may be seen. Necrosis is typically not evident. Sertoli cell tumours NOS are always unilateral. Tumours in patients with Peutz- Jeghers syndrome may be bilateral, and some large cell calcifying Sertoli cell tumours on record were also bilateral {1391}. Histopathology Tumour cells have oval, round, or elongated nuclei, and the nucleoli are not overtly prominent. Nuclear grooves and inclusions are usually not seen. The cytoplasm may be pale eosinophilic or clear and vacuolated due to lipids. In some instances the cytoplasm of tumour cells is prominently eosinophilic. Overall the cells appear bland and uniform. Mild nuclear pleomorphism and atypia is found in a minority of cases. Mitoses are uncommon and most cases contain fewer than 5 mitoses per ten high power fields. An increased number of mitotic figures (>5 per HPF) may be found in about 15% of cases, but in itself this finding should not be considered to be a sign of malignancy. The tumour cells are typically arranged into tubules surrounded by a basement membrane. These tubules may be solid or hollow with a central lumen. Furthermore, tumour cells may form retiform and tubular-glandular structures. Some tumours consist predominantly of solid sheets and nodules, but even in such neoplasms, well developed or abortive tubules are usually also present. The stroma between the tubules, cords and cell nests is fibrotic and moderately cellular to acellular and hyalinized. The hyalinized stroma contains often dilated blood vessels and may be markedly oedematous. Inflammatory cells are typically absent. Minor calcifications can be found in about 10% of cases, but occasional tumours may show more prominent deposits. Immunoprofile Sertoli cell tumours NOS stain with antibodies to vimentin (90%) and cytokeratins (80%) and to a variable extent with antibodies to inhibin (40%), and S100 Sex cord / gonadal stromal tumours 253
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 and 18: A B C Fig. 4.22 Seminoma. A Pseudog
Page 19 and 20: A B Fig. 4.27 Spermatocytic seminom
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: A B C Fig. 4.64 Leydig cell tumour.
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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