HIV-associated leiomyosarcoma - intracranial
Diagnosis certain
Updates to Case Attributes
Title
was changed:
HIV associated-associated leiomyosarcoma (intracranial)- intracranial
Presentation
was changed:
HIV +ve (CD 4+ count /, HAART, etc.. unknown).
Body
was changed:
The patient went on to have a craniotomy and resection.
Histology
Microscopic Description:
Sections show a spindle cell neoplasm. It is composed of irregularly arranged fascicles of spindle cells. Areas of microcystic change are noted. Occasional areas of necrosis are present. There are occasional mitotic figures.
Immunohistochemistry shows positive staining for smooth muscle actin. The tumour is negative for CD34, CD31, GFAP and S-100 protein. The tumour is positive for caldesmon.
Electron microscopy shows the tumour is comprisedcomposed of spindle cells with intermediate filaments. Many of these intermediate filaments undergo focal densifications, characteristic of smooth muscle cells. However, in addition, many of these tumour cells show fibronexus junctions, such as have been described in myofibroblasts.
FINAL DIAGNOSIS
This tumour was reviewed by Dr. [X], who hasa pathologist with expertise in soft tissue tumors. He feels, who felt that this is a malignant tumour and that it is a; more precisely, a leiomyosarcoma. He noted that such lesions have been described in patients infected with HIV and these are related to Epstein-Barr virus infection. He did immunohistochemical stains and these showed strong positive staining for caldesmon, a low MIB-1 index and strong positive staining throughout the lesion for Epstein-Barr virus in situ hybridization. There was negative staining for HHV8. These features would beare in keeping with a leiomyosarcoma associated with an Epstein-Barr virus infection in an immune-suppressed patient.
-<p><span style="line-height:1.6em">The patient went on to have a craniotomy and resection. </span></p><p><strong>Histology</strong></p><p>Microscopic Description:</p><p>Sections show a spindle cell neoplasm. It is composed of irregularly arranged fascicles of spindle cells. Areas of microcystic change are noted. Occasional areas of necrosis are present. There are occasional mitotic figures.</p><p>Immunohistochemistry shows positive staining for smooth muscle actin. The tumour is negative for CD34, CD31, GFAP and S-100 protein. The tumour is positive for caldesmon.</p><p>Electron microscopy shows the tumour is comprised of spindle cells with intermediate filaments. Many of these intermediate filaments undergo focal densifications, characteristic of smooth muscle cells. However, in addition, many of these tumour cells show fibronexus junctions, such as have been described in myofibroblasts.</p><p>FINAL DIAGNOSIS</p><p>Leiomyosarcoma </p><p>This tumour was reviewed by Dr. [X], who has expertise in soft tissue tumors. He feels that this is a malignant tumour and that it is a leiomyosarcoma. He noted that such lesions have been described in patients infected with HIV and these are related to Epstein-Barr virus infection. He did immunohistochemical stains and these showed strong positive staining for caldesmon, a low MIB-1 index and strong positive staining throughout the lesion for Epstein-Barr virus in situ hybridization. There was negative staining for HHV8. These features would be in keeping with a leiomyosarcoma associated with an Epstein-Barr virus infection in immune-suppressed patient.</p><p> </p>- +<p>The patient went on to have a craniotomy and resection. </p><p><strong>Histology</strong></p><p>Microscopic Description:</p><p>Sections show a spindle cell neoplasm. It is composed of irregularly arranged fascicles of spindle cells. Areas of microcystic change are noted. Occasional areas of necrosis are present. There are occasional mitotic figures.</p><p>Immunohistochemistry shows positive staining for smooth muscle actin. The tumour is negative for CD34, CD31, <a title="Glial fibrillary acid protein (GFAP)" href="/articles/glial-fibrillary-acid-protein-gfap">GFAP</a> and <a title="S-100" href="/articles/s100">S-100</a> protein. The tumour is positive for caldesmon.</p><p>Electron microscopy shows the tumour is composed of spindle cells with intermediate filaments. Many of these intermediate filaments undergo focal densifications, characteristic of smooth muscle cells. However, in addition, many of these tumour cells show fibronexus junctions, such as have been described in myofibroblasts.</p><p>FINAL DIAGNOSIS</p><p><a title="Leiomyosarcoma" href="/articles/leiomyosarcoma">Leiomyosarcoma</a></p><p>This tumour was reviewed by a pathologist with expertise in soft tissue tumors, who felt that this is a malignant tumour; more precisely, a leiomyosarcoma. He noted that such lesions have been described in patients infected with HIV and these are related to Epstein-Barr virus infection. He did immunohistochemical stains and these showed strong positive staining for caldesmon, a low MIB-1 index and strong positive staining throughout the lesion for Epstein-Barr virus in situ hybridization. There was negative staining for HHV8. These features are in keeping with a leiomyosarcoma associated with Epstein-Barr virus infection in an immune-suppressed patient.</p>
Diagnostic Certainty
was set to
.
References changed:
- 1. 1. Zevallos-Giampietri EA, Yañes HH, Orrego Puelles J, Barrionuevo C. Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis. (2004) Applied immunohistochemistry & molecular morphology : AIMM. 12 (4): 387-91. <a href="https://www.ncbi.nlm.nih.gov/pubmed/15536343">Pubmed</a> <span class="ref_v4"></span>
- 1. 1. Zevallos-Giampietri EA, Yañes HH, Orrego Puelles J, Barrionuevo C. Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis. (2004) Applied immunohistochemistry & molecular morphology : AIMM. 12 (4): 387-91. <a href="https://www.ncbi.nlm.nih.gov/pubmed/15536343">Pubmed</a> <span class="ref_v4"></span>
- 2. Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, Breeze RE, Greffe B, Lillehei KO, Stephens JK. Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. (1998) Human pathology. 29 (3): 240-5. <a href="https://doi.org/10.1016/s0046-8177(98)90042-9">doi:10.1016/s0046-8177(98)90042-9</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9496826">Pubmed</a> <span class="ref_v4"></span>
- 2. Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, Breeze RE, Greffe B, Lillehei KO, Stephens JK. Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. (1998) Human pathology. 29 (3): 240-5. <a href="https://doi.org/10.1016/s0046-8177(98)90042-9">doi:10.1016/s0046-8177(98)90042-9</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9496826">Pubmed</a> <span class="ref_v4"></span>
- 3. Gary W. Mierau, Brian S. Greffe, Douglas A. Weeks. Primary Leiomyosarcoma of Brain in an Adolescent with Common Variable Immunodeficiency Syndrome. (2009) Ultrastructural Pathology. 21 (3): 301-5. <a href="https://doi.org/10.3109/01913129709021926">doi:10.3109/01913129709021926</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9183831">Pubmed</a> <span class="ref_v4"></span>
- 3. Gary W. Mierau, Brian S. Greffe, Douglas A. Weeks. Primary Leiomyosarcoma of Brain in an Adolescent with Common Variable Immunodeficiency Syndrome. (2009) Ultrastructural Pathology. 21 (3): 301-5. <a href="https://doi.org/10.3109/01913129709021926">doi:10.3109/01913129709021926</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9183831">Pubmed</a> <span class="ref_v4"></span>
- 4. 4. Suankratay, Chusana, Shuangshoti, Shanop, Mutirangura, Apiwat, Prasanthai, Vichit, Lerdlum, Sukalya, Shuangshoti, Somruetai, Pintong, Jarupan, Wilde, Henry. Epstein-Barr Virus Infection-Associated Smooth-Muscle Tumors in Patients with AIDS. (2005) Clinical Infectious Diseases. 40 (10): 1521. <a href="https://doi.org/10.1086/429830">doi:10.1086/429830</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15844077">Pubmed</a> <span class="ref_v4"></span>
- Zevallos-Giampietri E.A., et al. Appl Immunohistochem Mol Morphol 2004; 12: 387-389.
- Zevallos-Giampietri EA, Yañes HH, Orrego Puelles J, Barrionuevo C. Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis. (2004) Applied immunohistochemistry & molecular morphology : AIMM. 12 (4): 387-91. <a href="https://www.ncbi.nlm.nih.gov/pubmed/15536343">Pubmed</a> <span class="ref_v4"></span>
- Kleinschmidt-DeMasters B.K., et al. Human Pathology 1998; 29: 240-245.
- Kleinschmidt-DeMasters BK, Mierau GW, Sze CI, Breeze RE, Greffe B, Lillehei KO, Stephens JK. Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. (1998) Human pathology. 29 (3): 240-5. <a href="https://doi.org/10.1016/s0046-8177(98)90042-9">doi:10.1016/s0046-8177(98)90042-9</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9496826">Pubmed</a> <span class="ref_v4"></span>
- Mieraw G.W., et al. Ultrastruct Pathol 1997; 21: 301-305.
- Gary W. Mierau, Brian S. Greffe, Douglas A. Weeks. Primary Leiomyosarcoma of Brain in an Adolescent with Common Variable Immunodeficiency Syndrome. (2009) Ultrastructural Pathology. 21 (3): 301-5. <a href="https://doi.org/10.3109/01913129709021926">doi:10.3109/01913129709021926</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/9183831">Pubmed</a> <span class="ref_v4"></span>
- Suankratay C, et al. Clin Infect Dis 2005; 40: 1521-1528.
Updates to Link Attributes
Title
was removed:
Type
was removed.
Visible
was set to
.
Updates to Primarylink Attributes
Updates to Study Attributes
Findings
was changed:
Enhancing left middle cranial fossa mass, with wide dural attachment.