CHAPTER X CHAPTER 4 - Cancer et environnement

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ICD-O codes Granulosa cell tumour 8620/1 Adult type granulosa cell tumour 8620/1 Juvenile type granulosa cell tumour 8622/1 Adult type granulosa cell tumour Incidence and clinical features This tumour is rare {1,477,1443,1705,1 812,2567}, grows slowly and only two dozen cases have been reported {1901}. Some are incidental. About 25% of patients have gynecomastia. The average age at presentation is 44 years (range, 16- 76 years). Patients have elevated serum levels of both inhibin, as occurs in other sex cord-stromal tumours {1781}, and Müllerian-inhibiting hormone, as occurs in similar ovarian tumours {1433}. Macroscopy These tumours are circumscribed, sometimes encapsulated, have a firm consistency and vary from yellow to beige. They vary from microscopic to 13 cm in diameter. The tumour surface may show cysts from 1-3 mm in diameter. Necrosis or haemorrhage are unusual. Histopathology Several patterns occur: macrofollicular, microfollicular, insular, trabecular, gyriform, solid and pseudosarcomatous. The microfollicular pattern is the most frequent. Microfollicles consist of palisading cells, which surround an eosinophilic material (Call-Exner bodies). Tumour cells are round to ovoid with grooved nuclei (coffee-bean nuclei) with one to two large peripheral nucleoli. Cellular pleomorphism and mitotic figures are infrequent, except for those areas showing fusiform cell pattern. The tumour may intermingle with seminiferous tubules and infiltrate the tunica albuginea. Some show focal theca cell differentiation, or have smooth muscle or osteoid {46}. Tumour cells are immunoreactive for vimentin, smooth muscle actin, inhibin, MIC2 (013-Ewing sarcoma marker), and focally cytokeratins. Fig. 4.78 Granulosa cell tumour, adult type. Juvenile type granulosa cell tumour This tumour is multicystic and its structure resembles that of Graafian follicles. Although it is rare, it is the most frequent congenital testicular neoplasm {1022, 2528}, comprising 6.6% of all prepubertal testicular tumours {1275}. Clinical features The tumour presents as a scrotal or abdominal asymptomatic mass, preferentially located in the left testis {1896}. It involves an abdominal testis in about 30% of cases. The contralateral testis is often undescended too. Most of the tumours are observed in the perinatal period, and presentation after the first year of life is exceptional. External genitalia are ambiguous in 20% and the most frequent associated anomaly is mixed gonadal dysgenesis, followed by hypospadias. In all cases with ambiguous genitalia the karyotype is abnormal: 45X / 46XY mosaicism or structural anomalies of Y chromosome. Neither recurrences nor metastases have been observed {400,2092,2136,2576,2895}. Neither gynecomastia nor endocrine disorders appeared associated. Macroscopy These tumours are usually cystic, with solid areas and partially encapsulated. The tumour size varies from 0.8 to 5 cm in size {1453}. Haemorrhage secondary to a torsion or trauma may make diagnosis difficult {407}. Histopathology Cysts are lined by several cell layers, depending on the degree of cystic dilation. The inner cells are similar to granulosa cells, while the outer cells resemble theca cells. Granulosa-like cells are Prognosis The tumour metastasizes in 20% or more of patients, even several years after the presentation {1223,1647}. Fig. 4.79 Juvenile granulosa cell tumour. Note prominent cysts. 256 Tumours of the testis and paratesticular
ound and have spherical, regularly outlined, euchromatic nuclei with inconspicuous nucleoli, and scanty, vacuolated cytoplasm. Occasionally, Call-Exner bodies are seen. Theca-like cells are elongated and show scanty cytoplasm and few mitoses. In some cases, the cystic fluid is mucinous. Occasionally, the tumour is seen within adjacent tubules {1905}. Ultrastructural examination reveals a dual epithelial smooth muscle cell differentiation {2048} and a similarity between the tumoural cells and both primitive Sertoli cells and preovulatory ovarian granulosa cells {2082}. Granulosa-like cells show diffuse immunostaining to vimentin, cytokeratins {956} and S-100 protein {2576}, and focal immunostaining to anti-Müllerian hormone {2180}. Theca-like cells immunoreact diffusely to vimentin, smooth muscle actin, and focally to desmin. The differential diagnosis is yolk sac tumour, and this can be addressed by immunostains {65,837,1651,2661}. Tumours of the thecoma / fibroma group Definition Tumours of the thecoma/fibroma group resemble their ovarian counterparts. Most intratesticular “thecomas” that have been reported are actually fibromas of gonadal stromal origin. Fibroma of gonadal stromal origin is a benign tumour, which displays fusiform cells and variable degrees of collagenization. ICD-O codes Thecoma 8600/0 Fibroma 8810/0 Synonyms Diffuse stromal form of gonadal stromal tumour {2592}, thecoma-like Sertoli cell tumour {482}, stromal tumour resembling fibroma {2547}, incompletely differentiated gonadal stromal tumour {1809}, testicular fibroma {1902}, testicular stromal tumour with myofilaments {932}, benign gonadal stromal tumour spindle fibroblastic type {64}, unclassified sex cord-stromal tumour with a predominance of spindle cells {2170}, myoid gonadal stromal tumour with epithelial differentiation {1904,2798}, theca cell tumour {2320}, and fibroma of gonadal stromal origin {1241}. Clinical features These tumours are rare, with only about 25 cases reported. The tumour presents as a slow growing, sometimes painful mass usually in the third and forth decades. It is not associated with hormonal alterations. Neither recurrences nor metastases have been observed. Macroscopy The tumour is a firm, well circumscribed, rarely encapsulated nodule, measuring 0.8 to 7 cm in diameter, and is yellow-white to white, without haemorrhage or necrosis. Histopathology Fusiform cells are arranged into fascicles or a storiform pattern, in slightly collagenized connective tissue with numerous small blood vessels. Cell density and amounts of collagen vary. Mitoses are usually scant, although up to four mitoses per high power field have been reported. Neither Sertoli cells nor granulosa cells are observed. Seminiferous tubules {571} with germ cells {2671} may be entrapped. Positive immunoreaction, to both vimentin, smooth muscle actin, and occasionally, to desmin, S-100 protein and cytokeratin have been observed. Inhibin and CD99 are non reactive. Tumour cells have ultrastructural features of both fibroblasts and myofibroblasts, although they are joined by desmosomes like Sertoli cells and granulosa cells {1726}. The differential diagnosis includes leiomyoma, neurofibroma, and solitary fibrous tumour {601}. Some malignant tumours such as primary testicular fibrosarcoma {2683} and stromal tumours should also be considered. Sex cord / gonadal stromal tumours: incompletely differentiated Definition Tumours composed largely of undifferentiated tissue in which abortive tubule formation, islands of Leydig cells, or evidence of other specific sex cord/gonadal stromal cell types are identified. These include tumours also recognizable as sex cord/gonadal stromal tumours but without specifically differentiated cell types. ICD-O code 8591/1 Histopathology Incompletely differentiated sex cord/gonadal stromal tumours are a heterogeneous group of testicular tumours that have been described under a variety of names but are not classifiable into more specific sex cord tumour types, including Leydig cell tumours, granulosa cell tumours and Sertoli cell tumours. Although heterogeneous, many of these tumours are similar {2170}, and are most often comprised of either short, wavy to round, spindle cells with nuclear grooves and a minor epithelioid component, or less commonly, long straight spindle cells with abundant cytoplasm, perinuclear vacuoles and blunt ended nuclei. Reticulin envelops aggregates of cells but not individual cells. Immunohistochemically, these tumours are most often reactive for both smooth muscle actin, and S-100 protein, a pattern also seen in both adult and juvenile granulosa cell tumours. Although most ovarian granulosa cell tumours are keratin positive, these tumours and most testicular granulosa cell tumours are keratin negative. Ultrastructural studies show desmosomes, numerous thin filaments, and focal dense bodies. Taken together these findings suggest granulosa cell differentiation in many of these incompletely differentiated tumours. With the exception of one large and poorly characterized tumour {1811}, the limited clinical followup available to date has been benign {932,2170,2860}. Sex cord / gonadal stromal tumours, mixed forms Definition The mixed form may contain any combination of cell types e.g. Sertoli, Leydig, and granulosa. Fig. 4.80 Sex cord stromal tumour of the testis. Sex cord / gonadal stromal tumours 257
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 and 36: A B C Fig. 4.64 Leydig cell tumour.
Page 37 and 38: A Fig. 4.69 Androgen insensitivity
Page 39: tinguished from Sertoli cell nodule
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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