Abdomen / Pelvis for Monday, April 7th, 2025

Contributed by University of California Davis
Liliane Gibbs, MD, MBA, Eric Diaz, MD, Sujit Jha, MD, and Rebecca Stein-Wexler, MD.

History

5-week-old male with acute right scrotal swelling, discoloration and inconsolable crying. History of bilateral congenital hydroceles at birth.

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Question

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Correct answer

Congenital Testicular Hemangioma

Discussion

This patient underwent a right partial orchiectomy and hydrocelectomy. The final pathology revealed congenital hemangioma with CD31, CD34 positivity and negative GLUT1 staining.

Testicular masses in neonates are rare and testicular hemangiomas are exceptionally rare. GLUT1 staining is essential for differentiating congenital hemangioma from infantile hemangioma, which has implication for prognosis and management.

In this case the initial concern was an inflammation/orchitis due to enlargement and heterogeneity of the right testicle with possible intratesticular abscess. The repeat ultrasound showed a hypervascular mass-like area within the testicle (images shown) which raised the question of congenital adrenal rest, malignant testicular tumor or congenital vascular malformation. 


Differential diagnosis

1- Orchitis: Is unusual in this case because of the focality of the vascularity.

2- Congenital Adrenal Rest Tumor (TART): Is a rare kind of benign tumor in the testis secondary to congenital adrenal hyperplasia (CAH), a hormonal disorder caused by hydroxylase deficiency. They are usually bilateral and present as hypervascular hypoechoic rounded masses located near the testicular mediastinum. The unilaterality and homogeneous isoechoic echogenicity were not typical in this case and the endocrine workup for congenital adrenal hyperplasia was negative.

3- Malignant testicular tumor such as germ cell tumors, stromal tumors and sex cord tumors: Ultrasound may show inhomogeneous, hypoechoic, and poorly defined masses. The mass in this case was well defined, isoechoic and relatively homogeneous, which is not typical for a malignant tumor. Tumor markers (e.g., beta-HCG, AFP, LDH) were negative.

4- Congenital Testicular Hemangioma: Are rare, benign vascular tumors present at birth, distinct from infantile hemangiomas as they are fully developed and don't grow after birth, and are characterized by negative GLUT1 staining, while showing CD31 and CD34 positivity. Although it progresses slowly, it can cause discomfort due to local pressure. Our patient presented with acute symptoms shortly after birth which may have been signs of intermittent torsion associated with the mass and the hydrocele.


References

  • Yu D, Lei Y. Testicular hemangioma: A case report. Urol Case Rep. 2017 Oct 7;16:20-21. doi: 10.1016/j.eucr.2017.09.012. PMID: 29021966; PMCID: PMC5633741. Zhang, Kaiping; Zhang, Yin; Chao, Min. Testicular hemangioma in a child: A rare case report and review of literature. Indian Journal of Pathology and Microbiology 66(1):p 222-225, Jan–Mar 2023. | DOI: 10.4103/ijpm.ijpm_881_21