Adrenal leiomyosarcoma in a young adult male

Nerli, Rajendra B; Ghagane, Shridhar C; Dixit, Neeraj S; Hiremath, Murigendra B; Deole, Sushant

doi:10.1007/s13691-019-00387-1

Cited by 6 publications

(15 citation statements)

References 11 publications

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“…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. Of the three specimens tested for NSE two were found positive (41,65) and one negative (44) and of the two tested for p53 one was found positive (39) and one moderately positive (43).…”

Section: Risk Factorsmentioning

confidence: 98%

“…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%

“…A total of 23.26% of the cases appeared extending to the IVC through tumor thrombus ( (34,38,51,53,63,65,73).…”

Section: Risk Factorsmentioning

confidence: 99%

“…Lujan et al reported the diagnosis of metastatic pulmonary carcinoma to the adrenal gland on a needle biopsy specimen and noted that the following "preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge" (63).…”

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).…”

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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Section: Risk Factorsmentioning

confidence: 98%

Section: Risk Factorsmentioning

confidence: 99%

“…A total of 23.26% of the cases appeared extending to the IVC through tumor thrombus ( (34,38,51,53,63,65,73).…”

Section: Risk Factorsmentioning

confidence: 99%

Section: Risk Factorsmentioning

confidence: 99%

Section: Risk Factorsmentioning

confidence: 99%

See 3 more Smart Citations

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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Challenges in the diagnosis of the enigmatic primary adrenal leiomyosarcoma: two case reports and review of the literature

Suzuki,

Takahashi,

Sugo

et al. 2023

BMC Endocr Disord

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Background Primary adrenal leiomyosarcoma is a rare and aggressive mesenchymal tumor derived from the smooth muscle wall of a central adrenal vein or its tributaries; therefore, tumors tend to invade the inferior vena cava and cause thrombosis. The great majority of tumors grow rapidly, which makes the disease difficult to diagnose in its early clinical stages and needs differentiation from adrenocortical carcinomas for the selection of chemotherapy including mitotane which causes adrenal insufficiency. Case presentation We presented two patients with adrenal leiomyosarcoma who were referred to our hospital with abdominal pain and harboring large adrenal tumors and inferior vena cava thrombosis. The endocrine findings, including serum catecholamine levels, were unremarkable. These two patients were considered clinically inoperable, and CT-guided core needle biopsy was performed to obtain the definitive histopathological diagnosis and determine the modes of therapy. The masses were subsequently diagnosed as primary adrenal leiomyosarcoma based on the histological features and positive immunoreactivity for SMA (smooth muscle actin), desmin, and vimentin. Conclusions Adrenal leiomyosarcoma derived from the smooth muscle wall of a central adrenal vein or its tributaries is rare but should be considered a differential diagnosis in the case of nonfunctioning adrenal tumors extending directly to the inferior vena cava. CT-guided biopsy is considered useful for histopathological diagnosis and clinical management of patients with inoperable advanced adrenal tumors without any hormone excess.

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Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics

Jabarkhel

Puttonen

Hansson

et al. 2020

Journal of the Endocrine Society

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Primary adrenal leiomyosarcoma (PAL) is a rare, high-grade proliferating mesenchymal tumor with a considerable risk of metastasis, deriving from the smooth muscle wall of a central adrenal vein, or its tributaries. Roughly 40 patients with PAL have been reported in the literature. Herein, we present 3 patients with incidentally discovered PAL, along with an overview of the current knowledge on the clinical, radiological, and histopathological characteristics of PAL.

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Adrenal leiomyosarcoma in a young adult male

Cited by 6 publications

References 11 publications

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

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

Challenges in the diagnosis of the enigmatic primary adrenal leiomyosarcoma: two case reports and review of the literature

Primary Adrenal Leiomyosarcoma: Clinical, Radiological, and Histopathological Characteristics

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