Multiple vascular leiomyomas involving bilateral adrenal glands, spleen, and epicardium, associated with bilateral testicular microlithiasis and empty sella turcica (original) (raw)
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Adrenal Leiomyoma: A case report
International Journal of Surgery Case Reports, 2021
Introduction and importance Adrenal Leiomyomas are infrequent tumors with only a few cases reported to date. They are difficult to differentiate from malignant adrenal tumors due to non-specific findings on clinical examination and imaging studies. Case presentation We discuss the case of a 49-year old male who had been experiencing generalized abdominal pain for 14 months and was found to have a mass on ultrasonography. Further evaluation with Contrast-enhanced Computerized Tomography (CECT) revealed an uneven soft tissue density mass in the retroperitoneal region of the left side. The histopathological examination of the excised mass was suggestive of a mesenchymal tumor, which was further confirmed as leiomyoma by immunohistochemistry. Clinical discussion Adrenal Leiomyomas are rare smooth muscle tumors that present with heterogeneously enhancing mass on radiologic imaging. On histopathological examination, spindle cells arranged in lobules and fascicles can be appreciated. Posit...
Testicular tumours of adrenogenital syndrome
Diagnostic Histopathology, 2009
Testicular 'tumour' of adrenogenital syndrome usually presents as bilateral testicular masses in patients with congenital adrenal hyperplasia. A thorough clinical evaluation is usually sufficient for diagnosis. In most circumstances this condition is well managed by replacement hormone therapy and infrequently requires surgical intervention with consequent histopathology evaluation. Attempt at differentiation from the morphologically similar Leydig cell tumour on biopsy tissue has potential for misdiagnosis; however, this distinction is crucial due to significantly different clinical interventions and the related medico-legal implications of orchidectomy. Through the following case, we illustrate these diagnostic difficulties, provide a summary from review of recent literature to assist in correct interpretation, and also stress the impact of either diagnosis.
Case Reports in Pathology, 2015
A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia (a.k.a. “testicular adrenal rest tumors” (TARTs) and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency (3BHSD) type, and started on treatment. Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untrea...
Bilateral testicular tumor in a patient with adrenogenital syndrome: A conservative approach
Pediatric Urology Case Reports, 2018
Testicular tumors of the adrenogenital syndrome (TTAGS) are a rare disease, with a benign curse. We present the case of a 12-year-old patient with congenital adrenal hyperplasia (CAH), precocious puberty and bilateral testicular masses. Hormonal control had been adequate. Incidental finding of bilateral testicular masses, with negative testicular tumoral biomarkers. The patient underwent right testicular sparing surgery, finding an intraparenchymal nodular mass. Pathological study showed a TTAGS, positive for CD99, Melan A, inhibin and synaptophysin markers. Conservative management was decided.
Congenital adrenal hyperplasia presented with bilateral testicular tumor: A case report
Caspian Journal of Internal Medicine, 2021
Background: Congenital adrenal hyperplasia (CAH) refers to group of congenital diseases resulting from impaired adrenal steroidogenesis, and its most common cause is 21hydroxylase deficiency. Testicular adrenal residual tumor (TART) is one of the major complications of CAH, possibly resulting from ectopic remnants of intra-testicular adrenal tissue which is stimulated by excessive secretion of adrenocorticotropic hormone (ACTH). This tumor can be misdiagnosed as Leydig cell tumor (LCT) in these people. Case Presentation: The patient we are presenting is a 20-year-old man with a history of precocious puberty and a height below 3% of the population who underwent radical left testicular orchiectomy with a complaint of bilateral testicular mass, which is reported LCT in the pathology report. In preoperative imaging examinations, bilateral adrenal hyperplasia is observed. In hormonal examinations, the patient is diagnosed with CAH and has been treated with corticosteroids for one year. Conclusion: In patients who present with bilateral testicular mass, it is the best image by abdominopelvic CT scan before surgery to detect CAH.
Leiomyoma of the testis: A rare testicular mass
Journal of Clinical …, 2008
We report the sonographic appearance of a testicular leiomyoma, which is a rare smooth muscle tumor of the testis. The patient presented with a painless testicular mass that was confirmed as an intratesticular tumor on sonographic examination. Histopathologic examination revealed a testicular leiomyoma. V V
Bilateral Adrenal and Testicular Mass in a Patient with Congenital Adrenal Hyperplasia
Acta Endocrinologica, 2019
Introduction. Congenital adrenal hyperplasia (CAH) autosomal recessive disorders characterized by impaired adrenal steroid hormone synthesis. The most common form is 21-hydroxylase deficiency (21OHD). Testicular adrenal rest tumors (TARTs) are benign intratesticular masses that occur in male patients with CAH. TARTs are quite common in patients with 21OHD who were diagnosed late. Case report. A 41-year-old male patient with CAH secondary to 21OHD. The patient was referred to our endocrinology department from the andrology clinic for bilateral adrenal masses. Bilateral orchiectomy had been performed due to bilateral testicular masses and azoospermia two years ago. The pathology was reported as Leydig cell tumor. In hormonal assessment, baseline cortisol levels were low, 17-hydroxyprogesterone levels with baseline and after cosyntropin stimulation test were high. As a result of clinic and laboratory assessment, the patient was diagnosed with simple virilising CAH due to 21OHD and adrenal insufficiency. Then, prednisolone replacement was initiated. Bilateral orchiectomy tissue blocks of the patient were reassessed and were considered TART. Magnetic resonance imaging revealed bilateral adrenal masses with 88x55 mm on the right and 41x22 mm on the left. Laparoscopic right adrenalectomy was applied and pathology was reported as myelolipoma. Follow-up of the mass on the left adrenal gland is ongoing. The patient is monitored under prednisolone and testosterone replacement therapy. Early diagnosis of CAH is very important because of the complications it causes. It should be considered especially for bilateral testicular and/ or adrenal masses. Both fertility and adrenal glands can be protected with an early diagnosis and an early glucocorticoid replacement.