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A rare metastatic site of primary adrenal leiomyosarcoma

Published:September 22, 2022DOI:https://doi.org/10.1016/j.amjms.2022.09.010

      Key terms

      CASE PRESENTATION
      A 56-year-old man was found to have a mass in the liver-kidney space during abdominal ultrasonography. His physical examination showed no positive signs. Plasma renin, cortisol, and aldosterone levels were within normal limits, while neuron-specific enolase (NSE) was estimated to be 16.53 ng/ml (reference range, 0–16.5 ng/ml); he tested negative for HIV. An abdominal computed tomographic (CT) scan revealed a lobular mass shadow of approximately 7.4 cm × 5.8 cm × 7.4 cm in size in the right adrenal gland area (Fig. 1A and 1B). The lesions were unclearly separated from the adjacent liver parenchyma; the adjacent inferior vena cava and right renal vein were found to be compressed (Fig. 1B, arrow). The lesion was removed by surgery. Microscopic examination revealed that the tumor cells (Fig. 1D, arrow) of the right adrenal gland were in the shape of a spindle with significant mitotic activity. The tumor was positive for desmin (Fig. E), smooth muscle actin (SMA) (Fig. F), and negative for CD117, S-100, DOG1, and pan-cytokeratin (PAN CK). The Ki-67 proliferation index was 30%. Based on these findings, the diagnosis of adrenal leiomyosarcoma was confirmed. At six months post-operation, a CT scan revealed a solid nodule on the right side of the abdominal wall in the patient (Fig. C arrow). The nodule was confirmed as a metastatic adrenal leiomyosarcoma using needle biopsy. Currently, the patient is undergoing radiotherapy.
      Primary adrenal leiomyosarcoma (PAL) is a rare malignant tumor of the mesenchymal tissue that originates from the smooth muscles of the inferior vena cava, central adrenal vein, and its branches.
      • Lack EE
      • Graham CW
      • Azumi N
      • et al.
      Primary leiomyosarcoma of adrenal gland. Case report with immunohistochemical and ultrastructural study.
      From 1981 to 2020, only 39 cases have been reported in the literature in the English language.
      • Wang Y
      • Teng Y
      • Na S
      • et al.
      Pleomorphic Leiomyosarcoma of the Adrenal Gland in a Young Woman: A Case Report and Review of the Literature.
      The clinical symptoms and laboratory examinations of PAL are non-specific, and the adrenal glands are located deep, making it difficult to diagnose early. The typical CT manifestations of primary adrenal leiomyosarcoma are: huge soft tissue masses in the adrenal gland, irregular in shape, typical in large patches or scattered small patches of necrosis, and peripheral blood vessels are easily affected. Most tumors have an abundant supply and show persistent enhancement. Primary adrenal leiomyosarcoma often needs to be differentiated from primary adrenal cortical carcinoma, adrenal adenoma, pheochromocytoma, gangliocytoma, etc. It has been reported that serum NSE levels could be a useful hallmark for the early detection of adrenal malignancy and its postoperative recurrence.
      • Goto J
      • Otsuka F
      • Kodera R
      • et al.
      A rare tumor in the adrenal region: neuron-specific enolase (NSE)-producing leiomyosarcoma in an elderly hypertensive patient.
      In this case, the NSE levels was slightly elevated. PAL usually metastasizes to the lungs, liver, and bones through the blood. However, in our case, it may be due to surgery that the tumor first metastasized to the abdominal wall.
      Preoperative diagnosis of PAL is difficult. The definitive diagnosis relies on histopathological and immunohistochemical examination. Surgical resection is currently the primary treatment method, and close follow-up is required after surgery.

      Source of Funding

      None.

      Declaration of competing interest

      None.

      References

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