CHAPTER X CHAPTER 4 - Cancer et environnement

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Sex cord / gonadal stromal tumours I.A. Sesterhenn J. Cheville P.J. Woodward I. Damjanov G.K. Jacobsen M. Nistal R. Paniagua A.A. Renshaw Sex cord / gonadal stromal tumours, pure forms Included in this category are Leydig cell tumours, Sertoli cell tumours, granulosa cell tumours and tumours of the thecoma/fibroma group. These tumours constitute about 4-6% of adult testicular tumours and over 30% of testicular tumours in infants and children. The name given to this group does not indicate a preference for any particular concept of testicular embryogenesis. As with the germ cell tumours, the aim throughout this section is to closely parallel the WHO terminology and classification of ovarian tumours. About 10% of these tumours, almost always in adults, metastasize. However, it may not be possible on histological grounds to forecast their behaviour. Some of these tumours occur in androgen insensitivity syndrome (AIS) and adrenogenital syndrome (AGS) and should be classified under tumour-like lesions. Leydig cell tumour Definition A tumour composed of elements recapitulating normal development and evolution of Leydig cells. ICD-O codes Leydig cell tumour 8650/1 Malignant Leydig cell tumour 8650/3 Fig. 4.61 Leydig cell tumour. Synonym Interstitial cell tumour. Epidemiology Leydig cell tumours account for 1-3% of testicular tumours {1318,1800,2664}. In infants and children, they constitute about 3% of testis tumours and 14% of stromal tumours {2366}. Unlike germ cell tumours, there is no race predilection {1800}. Occasionally, Leydig cell tumours are seen in patients with Klinefelter syndrome {1800,2664}. About 5-10% of patients have a history of cryptorchidism {1318}. Clinical features Signs and symptoms The tumour is most common in the 3 rd to 6 th decade and in children between 3 and 9 years {1318,2366}. Painless testicular enlargement is the most common presentation. Gynecomastia is seen in about 30% of patients either as a presenting feature or at clinical evaluation for a testicular mass {979,2664}. Libido A B Fig. 4.62 Leydig cell tumour. A Typical morphological appearance. B Leydig cell tumour. Note the Reinke crystals. and potency may be compromized. In children, precocious puberty is not uncommon {2831}. Leydig cell tumours produce steroids, particularly testosterone, androstenedione and dehydroepi-androsterone {298,2831}. Serum estrogen and estradiol levels may be elevated {828}. The latter may be associated with low testosterone and follicle stimulating hormone levels {213,1738}. Progesterone, urinary pregnanediol and urinary 17-ketosteroid levels may be elevated {535,2052}. Bilaterality is rare {1318,1800}. A Fig. 4.63 Leydig cell tumour. A Leydig cell tumour with cords of tumour cells. B Tumour cells stain intensely for inhibin, which is also present to a lesser extend in adjacent tubules. B Imaging Leydig cell tumours are generally well defined, hypoechoic, small solid masses but may show cystic areas, haemorrhage or necrosis. The sonographic appearance is quite variable and is indistinguishable from germ cell tumours. There are no sonographic criteria, which can differentiate benign from malignant Leydig cell tumours and orchiectomy is required. 250 Tumours of the testis and paratesticular tissue
A B C Fig. 4.64 Leydig cell tumour. A Note lipid rich cytoplasm. B Note lipomatous change. C Leydig cell tumour with adipose metaplasia. and not expansile growth pattern. Stromal tumours with prominent luteinization can mimic a Leydig cell tumour. The eosinophilic histiocytes of malakoplakia can be identified by the typical cytoplasmic inclusions (Michaelis Gutman bodies) and prominent intratubular involvement. A Fig. 4.65 Leydig cell tumour. A Leydig cell tumour with lipochrome pigment. B Unusual microcystic change in Leydig cell tumour. B Malignant Leydig cell tumour ICD-O code 8650/3 Macroscopy The tumours are well circumscribed, often encapsulated and 3-5 cm in size. The cut surface is usually homogeneously yellow to mahogany brown. There may be hyalinization and calcification. Expansion into paratesticular tissue can be detected in about 10-15% of cases {1318}. Histopathology The tumour shows variable histologic features recapitulating the evolution of Leydig cells. The most common type consists of medium to large polygonal cells with abundant eosinophilic cytoplasm and distinct cell borders. The cytoplasm may be vacuolated or foamy depending on the lipid content. Even fatty metaplasia can occur. Reinke crystals can be seen in about 30-40% of cases. The crystals are usually intracytoplasmic, but may be seen in the nucleus and interstitial tissue. Lipofuscin pigment is present in up to 15% of cases. Occasionally, the tumour cells are spindled or have scant cytoplasm. The nuclei are round or oval with a prominent nucleolus. There may be variation in nuclear size. Binucleated or multinucleated cells may be present. Some nuclear atypia can be observed. Mitoses are generally rare. The tumour has a rich vascular network as in endocrine tumours. The stroma is usually scant, but may be hyalinized and prominent. Occasionally it is oedematous. Psammoma bodies can occur {165,1739}. The growth pattern is usually diffuse, but may be trabecular, insular, pseudotubular and ribbon-like. Immunoprofile In addition to the steroid hormones, the tumours are positive for vimentin and inhibin {218,1159,1666,1727}. S100 protein has also been described {1663}. A positive reaction for cytokeratin does not exclude the diagnosis. Ultrastructure The polygonal Reinke crystals can have a variable appearance depending on the plane of sectioning e.g. various dot patterns, parallel lines, prismatic or hexagonal lattice {1290,2455,2456}. Differential diagnosis Most importantly, Leydig cell tumours have to be distinguished from the multinodular tumours of the adrenogenital syndrome. These are usually bilateral, dark brown and show cellular pleomorphism and pigmentation and are associated with a hyalinized fibrous stroma {1733,2230,2269}. Similar lesions are seen in Nelson syndrome {1234,1393}. Leydig cell hyperplasia has an interstitial Approximately 10% of Leydig cell tumours are malignant. Malignant features include large size (greater than 5 cm), cytologic atypia, increased mitotic activity, necrosis and vascular invasion {445,1318,1665}. The majority of malignant Leydig cell tumours have most or all of these features {445}. Most malignant Leydig cell tumours are DNA aneuploid and show increased MIB-1 proliferative activity, in contrast to benign Leydig cell tumours that are DNA diploid with low MIB-1 proliferation {445,1665}. On occasion, a benign Leydig cell tumour can be aneuploid. Currently, malignant Leydig cell tumours are managed by radical orchiectomy, and retroperitoneal lymphadenectomy. Malignant tumours do not respond to radiation or chemotherapy, and survival is poor with the majority of patients developing metastases that result in death. Fig. 4.66 Malignant Leydig cell tumour. Sex cord / gonadal stromal tumours 251
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: A B Fig. 4.59 Mixed germ cell tumou
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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