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CHAPTER X CHAPTER 4 - Cancer et environnement

CHAPTER X CHAPTER 4 - Cancer et environnement

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Fig. 4.115 Leiomyosarcoma. Coronal, T2-weighted,<br />

MR image shows a large h<strong>et</strong>erogeneous mass filling<br />

the left hemiscrotum and extending into the<br />

inguinal canal. It is displacing the base of the penis<br />

to the right (black arrow). A normal testis is seen<br />

within the right hemiscrotum (arrow).<br />

Fig. 4.116 Leiomyosarcoma of spermatic cord<br />

shows intersecting fascicles composed of atypical<br />

smooth muscle cells with blunt ended nuclei.<br />

Imaging<br />

Liposarcomas generally present as large<br />

extratesticular masses, which are often<br />

hyperechoic by ultrasound. However, the<br />

sonographic appearance of these<br />

tumours is variable and nonspecific. CT<br />

and MR imaging are much more specific<br />

with fat being easily recognized with both<br />

modalities {372,801}. By CT, fat will<br />

appear very low density similar to subcutaneous<br />

fat. On MR imaging the fat in a<br />

liposarcoma will follow the signal intensity<br />

of surrounding fat on all imaging<br />

sequences. Additionally a fat suppressed<br />

imaging sequence should be<br />

performed for confirmation. Fat will lose<br />

signal intensity (i.e. turn dark) on this<br />

sequence. Benign lipomas and hernias<br />

containing omentum are potential mimics,<br />

but lipomas are generally smaller<br />

and more homogeneous, and hernias<br />

are elongated masses, which can often<br />

be traced back to the inguinal canal.<br />

With the exception of liposarcoma, none<br />

of the other sarcomas can be differentiated<br />

from one another radiologically. They<br />

all tend to be large, complex, solid masses<br />

{372}. Because of their large size,<br />

their extent is b<strong>et</strong>ter demonstrated by CT<br />

and MR imaging rather than ultrasound.<br />

Histopathology<br />

Haemangiomas are classified according<br />

to the vessel type. Capillary and cavernous<br />

haemangiomas are most common<br />

within the scrotum, whereas angiokeratoma<br />

is the most common cutaneous vascular<br />

lesion {2578}. The latter features a<br />

superficial, dilated blood filled spaces initially<br />

associated with the epidermis, showing<br />

varying degrees of hyperkeratosis.<br />

Fibrous hamartoma of infancy is a subcutaneous<br />

lesion composed of streaks of<br />

fibroblasts, mature fat, and spherical clusters<br />

of primitive mesenchymal cells {2096}.<br />

Calcifying fibrous (pseudo)tumour is a<br />

densely collagenous, paucicellullar<br />

fibroblastic tumefaction that typically<br />

contains scattered psammomatous calcifications<br />

and a patchy lymphoplasmacytic<br />

infiltration.<br />

Granular cell tumours of the scrotum may<br />

be multifocal and are similar to those<br />

elsewhere in the skin.<br />

Leiomyomas are composed of mature<br />

smooth muscle cells. Larger tumours<br />

often have hyalinization, myxoid change<br />

and calcification. Some of these tumours<br />

arise from the tunica dartos {1886,2406}.<br />

Focal nuclear atypia may occur, but the<br />

presence of prominent atypia should<br />

lead to a careful search for mitotic activity<br />

or coagulation necrosis which are features<br />

of leiomyosarcoma.<br />

Leiomyosarcomas are typically composed<br />

of spindled cells with often elongated, blunt<br />

ended nuclei and variably eosinophilic,<br />

som<strong>et</strong>imes clumpy cytoplasm. Areas with<br />

round cell or pleomorphic morphology may<br />

Fig. 4.117 Paratesticular rhabdomyosarcoma.<br />

occur. The level of mitotic activity varies<br />

widely, but is often low.<br />

Male angiomyofibroblastoma-like tumour<br />

is grossly circumscribed, lobulated soft<br />

to rubbery mass. Distinctive at low magnification<br />

are prominent, large vessels<br />

with perivascular fibrinoid deposition or<br />

hyalinization. The tumour cells b<strong>et</strong>ween<br />

the vessels are tapered spindled cells<br />

with limited atypia, separated by fine collagen<br />

fibers. Focal epithelioid change is<br />

present in some cases. Nuclear palisading<br />

may occur, and a fatty component<br />

may be present; the latter has raised<br />

a question wh<strong>et</strong>her these tumours are<br />

fatty related neoplasms. Mitotic activity is<br />

very low. The tumour cells are immunohistochemically<br />

variably positive for<br />

desmin, muscle actins, CD34 and estrogen<br />

and progesterone receptors. This<br />

tumour is probably analogous to cellular<br />

angiofibroma as reported in females.<br />

Although some similarities with angiomyofibroblastoma<br />

of female genitalia have<br />

also been noted, these two processes<br />

are not considered synonymous {1442}.<br />

Aggressive angiomyxoma, a tumour that<br />

typically occurs in women, has been<br />

reported in men {1162,2649}. Our review<br />

of potential male cases in the AFIP files<br />

did not reveal any diagnostic examples<br />

of this entity. It seems likely that many<br />

tumours originally reported as male<br />

aggressive angiomyxomas, in fact, represent<br />

other entities, such as the male<br />

angiomyofibroblastoma-like tumour.<br />

Great majority of liposarcomas are well<br />

differentiated with various combinations<br />

of lipoma-like and sclerosing patterns.<br />

Presence of significant nuclear atypia in<br />

adipocytes is decisive. Multivacuolated<br />

lipoblasts may be present, but are not<br />

required for diagnosis. Dedifferentiation<br />

to spindle cell “fibrosarcoma-like” or pleomorphic<br />

“MFH-like” phenotype occurs in<br />

a proportion of paratesticular liposarco-<br />

Fig. 4.118 Embryonal rhabdomyosarcoma. Typical<br />

nuclear positivity for MyoD1.<br />

Tumours of paratesticular structures<br />

275

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