Scrotal Vascular Malformation

Ayodale SA Johnson Odulate, MD - Case Coordinator
Amy Fan-Yee Tsang, MD - Radiology Discussion
David W Kindelberger, MD - Pathology Discussion
Steven E Seltzer, MD - Attending Radiologist

September 22, 2003

Presentation

Imaging Findings

Sagittal and transverse ultrasound images of the left testicle show two abnormalities. The sagittal image shows an area of decreased echogenicity within the testicle. The transverse image shows a hydrocele (unrelated). The change in echogenicity indicates a 1.5 cm mass in the left testicle. The mass has heterogeneous echotexture, decreased compared to the normal appearance of the testicle. It is solid and there are no visible calcifications. It is fairly well defined on the sagittal view, though not exceptionally well circumscribed. Doppler ultrasound demonstrates flow both around and within the mass. Flow around the mass suggests hyperemia in the surrounding tissue.

Differential Diagnosis

Diagnosis

Discussion

The orchiectomy specimen weighed approximately 28 g. The normal tissue component measure approximately 4 cm. There is no discrete mass lesion. Instead, there are six areas of hemorrhage of various sizes scattered throughout the testicular parenchyma. The largest area measures approximately 1.5 cm. Low-power microscopy reveals a distorted architecture with normal (though compressed) tubular architecture on the right side of the image and multiple, thick-walled vascular spaces on the left side. At higher power, it is impossible to differentiate the types of vessels. The cells do not, however, appear to be neoplastic. The cells are all small, relatively uniform, and free of mitotic activity. The seminiferous tubules, though compressed, do not exhibit neoplastic features.

Radiology Discussion:

It is important to differentiate between intratesticular and extratesticular scrotal masses. Although extratesticular masses are almost always benign (hydrocele, varicocele, spermatocele, hernia), more than 95% of intratesticular masses are malignant. In fact, intratesticular masses should be considered malignant until proven otherwise. The differential diagnosis of a solid intratesticular mass includes germ cell tumor, stromal cell tumor, lymphoma, leukemia, metastasis (prostate, lung, melanoma, renal, and GI), carcinoid (primary, metastatic or associated with teratoma), sarcoidosis, inflammatory pseudotumor, hematoma, abscess, focal orchitis, and tuberculosis. There is also a long list associated with cystic intratesticular masses, though these are more rare: tubular ectasia of the rete testis, cystic dysplasia of the rete testis, non-neoplastic cysts (tunica albuginea cyst, intratesticular cyst), dermoid cyst, epidermoid cyst, cystic neoplasm (dermoid [benign/mature cystic teratoma], cystic lymphangioma, serous papillary cystic tumor, cystadenoma/adenofibroma of the rete testis), intratesticular spermatocele, intratesticular varicocele, hemorrhage, infarction, and necrosis. Due to the patient’s history of cryptorchidism, this case was somewhat expected to result in a diagnosis of testicular malignancy. References to the actual diagnosis, vascular malformation in the testicle, could not be found in a literature search. Therefore, this discussion focuses on cystic testicular masses. A brief, general discussion of vascular anomalies will follow.

Tubular ectasia of the rete testis typically occurs in older men (55+ years). It is often bilateral but asymmetric. There the cause may be congenital or mechanical (compression by tumor or chronic epididymitis), though it is also possible that ischemia or hormonal changes/imbalance could contribute. The end result is obliteration of efferent ductules. The salient features on ultrasound include multiple tubular or rounded anechoic cysts (variable sizes) within the mediastinum testes and/or an elongated shape that replaces the mediastinum. This mass is frequently associated with spermatoceles, testicular cysts, and epididymal cysts.

Cystic dysplasia of the rete testis is a developmental anomaly that is typically found in children. It is associated with ipsilateral renal agenesis in 41-55% of cases; it is thought to have an association with multicystic dysplastic kidney. A cryptorchid testis is seen in 35% of cases. Ultrasound imaging typically shows multiple thin-walled testicular cysts in an expanding lesion.

Tunica albuginea cysts are most common among men aged 40-60 years. They are generally palpable, due to a subcapsular location. They may be embryologic, traumatic, or post-infectious. These cysts are typically small, usually less than 10 mm. They occur in the upper lateral aspect of the testicle and are very firm. On ultrasound, they appear anechoic and may be septated.

Intratesticular cysts (another type of non-neoplastic cyst) typically occur in men over age 40. These masses are usually located near the rete testis and are not palpable. They vary in size from 2 - 18 mm, may be solitary or multiple, and are not firm. On ultrasound, they are anechoic with through transmission. Due to this appearance, they may be confused for a mature cystic teratoma; the latter, however, would be remote from the mediastinum.

The intratesticular varicocele is a newly defined entity that most closely resembles pseudoaneurysm. Patients may have pain related to passive congestion of the testis, which stretches the tunica albuginea. On ultrasound, is appears as multiple anechoic, tubular structures of varying sizes. Doppler imaging shows a venous flow pattern and venous spectral waveform; flow increases with the Valsalva maneuver.

Vascular anomalies that may affect the testes include hemangiomas and two types of malformation: high flow and low flow. Arteriovenous malformations (AVM) and arteriovenous fistulas (AVF) are both high low anomalies. Low flow anomalies include venous malformations, lymphatic malformations, capillary malformations (port wine stains), and capillary-lymphatic-venous malformations. MRI, ultrasound, and angiography may all be used to evaluate vascular malformations. T2-weighted, fat suppressed images are helpful in assessing the extent of an anomaly. GRE imaging can be used to differentiate between high- and low-flow lesions. The enhancement patterns revealed by T1-weighted pre- and post-contrast images can distinguish between the subtypes slow-flow lesions. Doppler ultrasound can be used to assess flow characteristics, while Doppler spectral analysis may help to distinguish arterial from venous flow. Angiography is the standard diagnostic modality for the evaluation of high-flow lesions (AVM, AVF), but has little or no diagnostic value in assessment of slow-flow anomalies (it may help to confirm the diagnosis).

References

Grunshaw ND, Gopichandran, TD. Case report: primary carcinoid tumor of the testis-ultrasound appearances. Clin Radiol 1993;47:290-1.

Robson WL, Thomason MA, Minette LJ. Cystic dysplasia of the testis associated with multicystic dysplasia of the kidney. Urology 1998; 51(3):477-9.

Walsh C, Rushton HG. Diagnosis and management of teratomas and epidermoid cysts. Urol Clinics of North America August 2000; 27:3.

Garrett JE, Cartwright PC, Snow BW, Coffin CM. Cystic testicular lesions in the pediastric population. J Urol March 2000; 163(3): 928-36.

Howe MC, Hatimy U, Husain J, Harney JV; McMahon RFT. Inflammatory pseudotumor of the testis. BJU International January 2001; 87(1).

Teefey, S. Sonography of the testis. www.Sonocredits.com

Friedland GW, et al. Uroradiology, an integrated approach. Edinburgh: Churchill Livingstone, 1982.

Light D. Testicle, malignant tumors. Emedicine. June 24, 2003. www.Emedicine.com

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