Primary Adrenal Leiomyosarcoma in an Arab Male: A Rare Case Report with Immunohistochemistry Study

Nagaraj, Veena; Mustafa, Mohammed; Amin, E.; Ali, Waleed; Sarsam, Shamil Naji; Darwish, Abdulla

doi:10.1155/2015/702541

Cited by 11 publications

(21 citation statements)

References 11 publications

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“…They are usually found in image studies as large heterogeneous masses with no specific characteristics, which makes them undistinguishable from other adrenal tumors (3). The pathogenesis of adrenal leiomyosarcoma remains unknown, but there might be a correlation with Epstein-Barr Virus infection in patients with acquired immunodeficiency syndrome (AIDS) (4). The treatment of choice is surgical resection with negative margins, after the evaluation of the tumor secretion to exclude the diagnosis of a functional tumor.…”

Section: Discussionmentioning

confidence: 99%

Primary adrenal leiomyosarcoma: A case report

et al. 2020

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Adrenal leiomyosarcomas are rare mesenchymal tumors of the suprarenal region that are usually diagnosed after they have reached a large size. We report the case of a 62-year-old male with an incidentally found left adrenal mass. Magnetic resonance imaging of the retroperitoneal space showed a heterogeneously enhanced mass, measuring 10x8.2 cm, with characteristics suspicious of malignancy. The patient underwent left radical adrenalectomy after the hormonal evaluation of the tumor due to the high probability of adrenocortical carcinoma. However, microscopic examination of the tumor showed a spindle cell sarcoma. Immunohistochemically the neoplastic cells were found positive for desmin and smooth muscle actin and the diagnosis of a well differentiated adrenal leiomyosarcoma was established. During follow-up the patient presented an aggressive course as he developed bone, liver and pulmonary metastases early postoperatively, which were treated with radiation therapy and chemotherapy. The patient has progressive metastatic disease while on chemotherapy 31 months after surgery.

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Section: Discussionmentioning

confidence: 99%

Primary adrenal leiomyosarcoma: A case report

et al. 2020

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“…One case had a known celiac disease and a history of laparotomies due to intestinal obstruction (37), one patient was a hepatitis B virus (HBV) carrier ( 68) and one case had a history of mediastinal seminoma at the age of 10, treated with RT to the chest and testes (38). (31,32,40,46,49,52,63,64), in 2 cases the mass was described as having irregular borders (48,49) and in 1 as lobulated (57).…”

Section: Risk Factorsmentioning

confidence: 99%

“…The median surgical specimen's size measured was 10.5 cm (average=11.22 cm, minimum=0.8 cm, maximum=27 cm) out of 27 unilateral (37, 39, 40, 43-45, 47, 49, 50, 52, 54-58, 60-62, 64-67, 69-73) and 3 autopsy specimens (3, 59) that provided size and weighted in median 482 g (average=692 g, minimum=47 g, maximum=2400 g) out of 10 unilateral tumors that provided weight (37,39,40,47,50,52,56,70,72,73), 3 cases that provided the weight of the surgical specimen containing en bloc the resected kidney were not included (43)(44)(45).…”

Section: Risk Factorsmentioning

confidence: 99%

“…From the 27 surgical pathology and one autopsy report (59) that commented on the presence of normal adrenal tissue, 19 (63). Both specimens tested for calponin were found positive (50, 65), and both negative for calretinin (52,73), three that were tested for myoglobin were found negative (46,65,71), three specimens tested for myogenin were found negative (43,65,70) and one positive (49). Of the 19 specimens tested for cytokeratin 17 were found negative (34, 42, 43, 46, 47, 50, 52, 54-56, 64-66, 70-73), one was patchy positive (32) and one was found positive for CK Mak6 (51).…”

Section: Risk Factorsmentioning

confidence: 99%

“…Of the 19 specimens tested for cytokeratin 17 were found negative (34, 42, 43, 46, 47, 50, 52, 54-56, 64-66, 70-73), one was patchy positive (32) and one was found positive for CK Mak6 (51). Of 6 specimens tested for inhibin (37,49,50,52,62,70) and of the 7 for chromogranin (44, 51, 52, 65, 70, 72, 73) all were found negative. Of the five specimens tested for synaptophysin (44,52,70,72,73) and for c-kit (38,42,63,64,67) all were found negative.…”

Section: Risk Factorsmentioning

confidence: 99%

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Review of the Literature on Leiomyoma and Leiomyosarcoma of the Adrenal Gland: A Systematic Analysis of Case Reports

Σακελλαρίου

Dellaportas

Peppa

et al. 2020

In Vivo

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Background/Aim: To date few cases of smooth muscle-derived tumors of the adrenal gland have been reported and their treatment remains a medical challenge. The aim of this manuscript was to systematically review the literature and present the tumor characteristics and their management in order to provide a standardized approach to their diagnosis and management. Materials and Methods: We searched five databases (PubMed, Scopus, Elsevier, ResearchGate, Google scholar) for relevant articles published until March 2020. Results: Twenty-two cases of adrenal leiomyoma, four cases of adrenal smooth muscle tumor and forty-five cases of adrenal leiomyosarcoma were included. Conclusion: We present the demographic, clinical, radiological, pathological and oncological characteristics and prognosis of tumors of the adrenal gland arising from smooth muscle cells, as well as describe the common clinical investigations and therapeutic modalities that have been reported as part of their management.Adrenal tumors can be detected either incidentally or after investigation of signs and symptoms that they evoke. Generally, they can be histologically categorized as primary tumors of the adrenal cortex or medulla, metastases from other primary malignancies and other tumors of the adrenal gland which originate from connective tissue cells. Adrenal cortical tumors are comprised by (asymmetric) adrenal cortical hyperplasia, adenoma, myelolipoma, adrenocortical carcinoma, oncocytic adrenocortical neoplasms; medullary tumors are comprised by pheochromocytoma, ganglioneuroma, neuroblastoma, ganglioneuroblastoma and teratoma. Connective tissue origin tumors are rarer and are comprised mainly by haemangioma and lymphangioma with a few cases of leiomyoma, lipoma, hemangiosarcoma, lymphoma, leiomyosarcoma and liposarcoma, as reported in the literature. Patients with primary malignancies in lung, breast, kidney, colon, oesophagus, pancreas, liver, stomach or with melanoma can present with adrenal metastases spread from these tumors, which can commonly be found bilaterally (1).Leiomyomas (LM) and leiomyosarcomas (LMS) are mesenchymal tumors that derive from smooth muscle cells. Their benign -leiomyoma or malignant -leiomyosarcoma characterization depends on nuclear atypia and/or pleomorphism, mitotic activity and tumor cell necrosis (2). Leiomyomas and leiomyosarcomas of the adrenal gland comprise a very small percentage of the gland's pathology with very little data available in the literature. The aim of this manuscript was to systematically review the literature and present demographic, clinical, radiological, pathological and oncological characteristics and prognosis of tumors of the adrenal gland arising from smooth muscle cells, as well as describe the common clinical investigations and therapeutic modalities that have been reported as part of their management.

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Pleomorphic spindle cell sarcoma of the adrenal gland: an extremely rare cause of a large abdominal mass and a challenging diagnosis

Ozaal

Rajendra

2022

Journal of Surgical Case Reports

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Primary adrenal pleomorphic spindle cell sarcoma (PSCS) is an exceedingly rare mesenchymal tumour that was previously known as malignant fibrous histiocytoma. It commonly occurs in extremities, retroperitoneum, peritoneal cavity and rarely in visceral organs. We report the first case of PSCS in the left adrenal gland in a 65-year-old female who presented with a large abdominal mass with vague symptoms. The wide range of differential diagnoses posed a challenge in establishing the diagnosis. However, this was overcome by appropriate radiological, intra-operative, histological and most importantly, comprehensive immunohistochemical findings. The patient underwent complete surgical resection of the tumour and had an unremarkable recovery. She remains without metastasis or recurrences to date through her 18 months of post-operative follow-up despite the poor prognosis of this tumour.

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Primary Adrenal Leiomyosarcoma in an Arab Male: A Rare Case Report with Immunohistochemistry Study

Cited by 11 publications

References 11 publications

Primary adrenal leiomyosarcoma: A case report

Primary adrenal leiomyosarcoma: A case report

Review of the Literature on Leiomyoma and Leiomyosarcoma of the Adrenal Gland: A Systematic Analysis of Case Reports

Pleomorphic spindle cell sarcoma of the adrenal gland: an extremely rare cause of a large abdominal mass and a challenging diagnosis

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