Primary Leiomyosarcoma of the Adrenal Gland

Etten, Boudewijn van; Ijken, M G A van; Mooi, Wolter J.; Oudkerk, Matthijs; Geel, Albertus N. van

doi:10.1155/s1357714x01000184

Cited by 20 publications

(29 citation statements)

References 13 publications

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“…Twenty cases have been reported in the English literature since 1981. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Typically, the disease presents as a symptomatic retroperitoneal mass more than 10 cm in diameter. It can be associated with a mass effect on surrounding organs, involvement of the inferior vena cava (IVC) by a tumor thrombus, local organ invasion or distant metastasis.…”

Section: Introductionmentioning

confidence: 99%

Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

Schwaab¹,

Azzouni²,

Azabdaftari³

2012

N A J Med Sci

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Primary adrenal leiomyosarcomas are rare and usually symptomatic at presentation. The presentation of a large adrenal mass should raise suspicion of adrenal leiomyosarcoma as a differential diagnosis. To our knowledge, primary adrenal leiomyosarcoma has been reported in 20 cases in the English literature. Here we describe a case of primary adrenal leiomyosarcoma in a 76-year-old Caucasian female. The patient complained of right upper quadrant abdominal pain for 2 months. Contrast-enhanced magnetic resonance imaging showed a heterogeneous 10.5 cm adrenal mass with a suspected inferior vena cava tumor thrombus without lymph node enlargement or distant metastasis. The patient underwent a right adrenalectomy, partial resection of the inferior vena cava and reconstruction of the inferior vena cava with a pericardial patch. Histopathologic examination was consistent with leiomyosarcoma. At 3 months postoperatively, a follow-up computed tomography scan of the chest, abdomen and pelvis without intravenous contrast was done that showed multiple bilateral pulmonary metastatic lesions, bilateral hilar and mediastinal lymphadenopathy, liver metastasis, a new mass at the head of the pancreas, and a new mass at the lower pole of the right kidney. The patient was deemed to be unfit for systemic chemotherapy, and was referred to the hospice service for palliative care. The patient died 4 months after surgery.

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Section: Introductionmentioning

confidence: 99%

Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

Schwaab¹,

Azzouni²,

Azabdaftari³

2012

N A J Med Sci

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“…Given the few cases reported in the literature, disease progression and prognosis are difficult to estimate. Tumor size does not seem to be a good predictor of morbidity, as suggested by Etten et al (18), who reported a patient with a 27-cm adrenal leiomyosarcoma without evidence of metastasis on autopsy. Ferrario et al (9) postulated that morphologic grading (presence of mitotic activity, necrotic areas, nuclear atypia) can be predictors of survival.…”

Section: Discussionmentioning

confidence: 88%

Recurrent Primaty Adrenal Leiopyosarcoma: A Complete Literature Review and Presentation of a Rare Adrenal Tumor

Manzano

Nosé

Fernandez-Castro

et al. 2015

AACE Clinical Case Reports

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Objective: Leiomyosarcomas are mesenchymal tumors that very rarely present as a primary adrenal malignancy. Methods: We present the complete clinical presentation, imaging, and anatomicopathologic features of a unique case of recurrent primary adrenal leiomyosarcoma (PAL) after initial surgery. This case is accompanied by an updated literature review. Results: A 63-year-old Hispanic female referred to our clinic 4 months after having right adrenalectomy. Eight months prior to this encounter, she started to notice bilateral lower extremity edema with skin changes. Computed tomography of the abdomen revealed a large 6.8 × 4.4 cm adrenal mass that appeared to be encroaching in the inferior vena cava with hypodense areas, irregular borders, and a necrotic center. A preoperative hormonal evaluation failed to reveal evidence of a functional adrenal tumor. Microscopic examination revealed spindle cell neoplasia. Immunohistochemistry of the surgical specimen revealed positive staining for desmin, smooth muscle actin, and focal myogenin (MYF-4). S-100 protein and inhibin were negative. The diagnosis of intermediate-grade leiomyosarcoma was confirmed. Two months after surgery, surveillance positron emission tomography scan revealed another right adrenal mass measuring 5.6 × 5 cm, with a standardized uptake value of 2.1. Conclusion: PAL is excessively rare. To our knowledge, this is only the second case of PAL with documented recurrence and the 19th case reported in the current medical literature. Surgical removal of the tumor is the first step in treatment. Adjuvant therapies are still not well standardized based on the low incidence of cases.

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“…Some studies have reported that radiation therapy is particularly important in cases where a large tumor locally redevelops or a tumor is located at a site that makes its complete removal difficult [12][13][14]. Doxorubicin and ifosfamide have been the most effective chemotherapy for soft tissue sarcomas in monotherapy and combination therapy for the past 4-5 years.…”

Section: Discussionmentioning

confidence: 99%

“…Radiotherapy was not used in this case, but its use in cases of localized recurrence has been reported to be effective. It is possible that its use in addition to chemotherapy should also have been considered in the treatment of our patient [12][13][14].…”

Section: Discussionmentioning

confidence: 99%

Primary Adrenal Leiomyosarcoma in an Aged Japanese Woman: A Rare Case Report

Aoki¹,

Tanaka²,

Suzuki³

et al. 2017

J Clin Case Rep

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Primary adrenal leiomyosarcoma is an extremely rare tumor. Here, we report the case of a primary adrenal leiomyosarcoma in an 81-year-old woman. Computed tomography showed a right adrenal tumor (7 cm). There were no abnormal findings on physical examination and her serum adrenal hormone levels were normal. However, 67Gallium scintigraphy showed gallium accumulation at the site of a right adrenal tumor, and malignancy was suspected. The right adrenal tumor was surgically removed and histological examination revealed that it was a leiomyosarcoma. After surgical treatment, the patient was administered different types of chemotherapy but she died of local recurrence 1 year later.

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Primary Leiomyosarcoma of the Adrenal Gland

Cited by 20 publications

References 13 publications

Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

Primary Adrenal Leiomyosarcoma: Case Report and Review of Literature

Recurrent Primaty Adrenal Leiopyosarcoma: A Complete Literature Review and Presentation of a Rare Adrenal Tumor

Primary Adrenal Leiomyosarcoma in an Aged Japanese Woman: A Rare Case Report

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