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CASE REPORT: Bilateral Adrenal Leiomyosarcoma in an Immunocompromised Pediatric Patient: A Case Report after 7 Years of Followup

By: J. Antonio Grandez-Urbina, MD, MSc(c); J. Eduardo Tejeda Mariaca, MD; L. Toledo-Luksic, MD; Mario J. Valladares-Garrido, MD; Campos Santolalla, MD | Posted on: 01 Mar 2022


Leiomyosarcoma is a very rare tumor that originates in the smooth muscle. These tumors can develop in the central nervous system, intestine, liver, skin, lungs, larynx and pharynx, very rarely originating in the adrenal glands. There are few reports of this type of tumor, especially in a bilateral presentation.1

Although the pathogenesis of adrenal leiomyosarcoma is not fully understood, it has been observed that immunosuppression may predispose itself to this occurrence.2

We present the case of a pediatric patient immunosuppressed by HIV who was diagnosed with bilateral adrenal leiomyosarcoma and was followed for 7 years after surgical treatment. This is the first reported case in which long-term followup has been performed in such a young patient.

Clinical Case

A 10-year-old female admitted to Alberto Sabogal Sologuren Hospital, Callao, Peru, presented with dyspnea on moderate exertion, mild abdominal pain, nausea and liquid stools, associated with signs of chronic malnutrition. Ancillary tests were performed, and HIV infection was diagnosed, initiating retroviral treatment.

Physical examination revealed multiple anterior and posterior cervical adenopathies (approximately 1.5 cm in diameter), the abdomen was excavated with slight pain on palpation, with no evidence of visceromegaly.

Laboratory tests showed an HIV viral load of 91,406 copies/ml; CD4 count: 237 cell/μL; positive anti-herpes virus 1 antibody (IgG), negative anti-herpes virus 1 antibody (IgM), negative anti-herpes virus 2 antibody IgG and IgM; positive Epstein-Barr virus IgG antibodies, negative IgM antibodies. Serum cortisol and dehydroepiandrosterone sulfate were normal. The remainder of laboratory investigations were normal.

“Although the pathogenesis of adrenal leiomyosarcoma is not fully understood, it has been observed that immunosuppression may predispose itself to this occurrence.”

Multislice computerized tomography and magnetic resonance imaging of the abdomen and pelvis with contrast showed heterogeneous solid masses with delimited borders in bilateral adrenal glands, 5.3 × 5.4 cm on the right and 3.7 × 3.3 cm on the left. No local invasion or free fluid in the peritoneal cavity was observed (figs. 1 and 2).

Bilateral adrenalectomy was performed using an open transabdominal approach through a suprainfraumbilical incision to facilitate immediate access and control of the inferior vena cava (fig. 3). The patient remained in pediatric intensive care until recovery with hydrocortisone replacement therapy.

In the pathology study, spindle cells with low atypia were evidenced. The tumor showed a positive immunohistochemical reaction for vimentin and alpha-smooth-muscle-actin (αSMA), negative for s100 and CD68. This confirmed the diagnosis of low-grade leiomyosarcoma.

Figure 1. Computerized tomography in axial section with contrast. Solid masses with delimited borders are shown in bilateral suprarenal cells, 6 cm on the right and 3 cm on the left. It presents soft tissue density and heterogeneous enhancement on contrast.
Figure 2. Nuclear magnetic resonance, turbo spin echo T2 sequence with fat suppression, pre- and postcontrast. Heterogeneous neoformative lesions in both adrenal glands with mass effect but without local invasion.
Figure 3. Bilateral adrenal tumor.
“The age range of presentation of primary adrenal leiomyosarcoma is very wide; however, it is extremely rare in the pediatric age group.”

The patient presented a favorable evolution in the postoperative period. She continued with retroviral treatment and replacement therapy, maintaining undetectable viral loads. After 7 years of followup by the infectious disease service, urology and pediatric oncology departments, there have been no signs of recurrence.


Primary adrenal leiomyosarcoma is an exceptionally rare malignant neoplasm arising from mesenchymal tissue. It is thought to arise from the smooth muscle of the central adrenal vein, accounting for only 0.1% to 0.2% of all retroperitoneal sarcomas. Fewer than 50 cases of adrenal leiomyosarcoma have been described in the literature to date.1

There is evidence that this malignant tumor is associated with HIV immunodeficiency and Epstein-Barr virus infection. However, so far there is no certainty as to the pathogenic mechanism.2,3

The age range of presentation of primary adrenal leiomyosarcoma is very wide; however, it is extremely rare in the pediatric age group. There is only 1 report by Linos et al in which the authors presented the case of a 14-year-old patient diagnosed with bilateral primary adrenal leiomyosarcoma immunosuppressed by HIV.4 Unlike our case, this patient did not present coexistent infection with Epstein-Barr virus.

The clinical picture of adrenal leiomyosarcoma is very nonspecific. Most cases present with abdominal pain and weight loss. Peripheral edema may also occur due to invasion of the vena cava.

Imaging tests are very important in patients with suspected adrenal tumor, as they allow determination of the extent and possibility of surgical resection. Computerized tomography and magnetic resonance imaging show heterogeneous lesions with occasional central necrosis. However, the imaging characteristics do not differ from other adrenal lesions.1

The diagnosis of leiomyosarcoma is confirmed by immunohistochemical studies of the tumor mass. The majority of leiomyosarcomas are positive for αSMA, muscle-specific-actin (HHF35), vimentin and desmin. Since these are retroperitoneal tumors, neuroendocrine tumors, adrenocortical carcinoma and melanoma should be ruled out by immunohistochemical study.2,5

“Imaging tests are very important in patients with suspected adrenal tumor, as they allow determination of the extent and possibility of surgical resection.”

Treatment of adrenal leiomyosarcoma is based on radical surgical resection. There are reports of laparoscopic adrenalectomies with good results. However, there is no evidence to define the best surgical approach for this type of tumor. For large tumors or with suspected infiltration of neighboring organs, open surgery may be a good option. In our case, an open bilateral adrenalectomy was performed with a very good postsurgical evolution and satisfactory oncologic results.2,4,5

According to the literature, the survival rate for patients with adrenal leiomyosarcoma at 2 years is 15.3%.2 In our case, after 7 years of followup, the patient is free of recurrence in subsequent tomographic controls. This represents the case with the longest followup reported to date.


Leiomyosarcoma of the adrenal gland is a very rare tumor that can occur in pediatric patients. It is important to consider it as a differential diagnosis in patients with HIV infection and Epstein-Barr virus associated with adrenal lesions. Radical surgical treatment is the key to improve survival. The definitive diagnosis is based on pathological anatomy and immunohistochemistry. Survival in patients with adrenal leiomyosarcoma is very poor. However, the absence of metastases, complete resection of the lesions, associated with low atypia, as presented in our case, may improve survival. Further studies are required to define a better management protocol in this type of rare tumor.


We thank the Pediatrics, Urology and Anatomic Pathology Departments of HNASS.

  1. Jabarkhel F, Puttonen H, Hansson L et al: Primary adrenal leiomyosarcoma: clinical, radiological, and histopathological characteristics. J Endocr Soc 2020; 4: bvaa055.
  2. Sakellariou M, Dellaportas D, Peppa M et al: Review of the literature on leiomyoma and leiomyosarcoma of the adrenal gland: a systematic analysis of case reports. In Vivo 2020; 34: 2233.
  3. Zhou Y, Tang Y, Tang J et al: Primary adrenal leiomyosarcoma: a case report and review of literature. Int J Clin Exp Pathol 2015; 8: 4258.
  4. Linos D, Kiriakopoulos AC, Tsakayannis DE et al: Laparoscopic excision of bilateral primary adrenal leiomyosarcomas in a 14-year-old girl with acquired immunodeficiency syndrome (AIDS). Surgery 2004; 136: 1098.
  5. Wei J, Sun A, Tao J et al: Primary adrenal leiomyosarcoma. Int J Surg Pathol 2014; 22: 722.