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An Unusual Presentation of Isolated Leptomeningeal Disease in Carcinoma of Unknown Primary With Pancreatic Features.

Anne M, Ahmad N, Lee P, Aziz M, Lebowicz Y - J Investig Med High Impact Case Rep (2013)

Bottom Line: A 45-year-old woman was found with mediastinal and abdominal lymphadenopathy with lymph node biopsy consistent with adenocarcinoma, expressing immunomarkers CK7, CK20, and Ca19-9 along with markedly elevated serum Ca19-9 level.She was then noted to have slowly rising Ca19-9 level that did not correlate with her lack of evidence of systemic disease progression.Eventually, she presented with neurologic symptoms and was found on imaging to have isolated LMD.

View Article: PubMed Central - PubMed

Affiliation: Hofstra North Shore-Long Island Jewish School of Medicine, New Hyde Park, NY, USA.

ABSTRACT
Leptomeningeal disease (LMD) can occur in a small percentage of patients with active metastatic cancer. However, we report a case of LMD occurring during disease remission in a patient with carcinoma of unknown primary with panreaticobiliary features. A 45-year-old woman was found with mediastinal and abdominal lymphadenopathy with lymph node biopsy consistent with adenocarcinoma, expressing immunomarkers CK7, CK20, and Ca19-9 along with markedly elevated serum Ca19-9 level. The patient was started on a pancreatic cancer directed chemotherapy regimen of Folfirinox (5-fluorouracil, leucovorin, oxaliplatin, irinotecan) and achieved complete response. She was then noted to have slowly rising Ca19-9 level that did not correlate with her lack of evidence of systemic disease progression. Eventually, she presented with neurologic symptoms and was found on imaging to have isolated LMD.

No MeSH data available.


Related in: MedlinePlus

Saggital T1-weighted postcontrast magnetic resonance image of brain demonstrating diffuse leptomeningeal enhancement involving both cerebellar hemispheres, the superior cerebellar vermis, and parieto-occipital lobes, as indicated by the red arrows.
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fig3-2324709613494830: Saggital T1-weighted postcontrast magnetic resonance image of brain demonstrating diffuse leptomeningeal enhancement involving both cerebellar hemispheres, the superior cerebellar vermis, and parieto-occipital lobes, as indicated by the red arrows.

Mentions: She was subsequently noted to have a slowly rising Ca19-9 level, but without any symptoms or evidence of systemic disease progression on repeat radiographic imaging. After 12 months from initial diagnosis, the patient was brought to the emergency department with sudden onset of headaches, slurred speech, generalized weakness, and agitation. At this point, the Ca19-9 level was 1920 U/mL. Initial CT of the head with and without contrast showed evidence of leptomeningeal enhancement involving the posterior fossa (Figure 1). Analysis of cerebrospinal fluid (CSF) obtained via lumbar puncture revealed mildly elevated protein level of 50 mg/dL (reference range = 15-45 mg/dL), with normal glucose level of 67 mg/dL (reference range = 40-70 mg/dL). Cytological examination of CSF demonstrated malignant cells that were morphologically and immunohistochemically identical to the original biopsy from the periaortic lymph node metastasis (Figure 2). Magnetic resonance imaging of the brain and spine was consistent with abnormal leptomeningeal enhancement involving bilateral cerebellar hemispheres, vermis, parieto-occipital lobes, as well as diffuse enhancement of the spine (Figures 3 and 4). Treatment with intrathecal chemotherapy was discussed; however the family opted for palliative options and the patient rapidly deteriorated and expired.


An Unusual Presentation of Isolated Leptomeningeal Disease in Carcinoma of Unknown Primary With Pancreatic Features.

Anne M, Ahmad N, Lee P, Aziz M, Lebowicz Y - J Investig Med High Impact Case Rep (2013)

Saggital T1-weighted postcontrast magnetic resonance image of brain demonstrating diffuse leptomeningeal enhancement involving both cerebellar hemispheres, the superior cerebellar vermis, and parieto-occipital lobes, as indicated by the red arrows.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License 1 - License 2 - License 3
Show All Figures
getmorefigures.php?uid=PMC4528790&req=5

fig3-2324709613494830: Saggital T1-weighted postcontrast magnetic resonance image of brain demonstrating diffuse leptomeningeal enhancement involving both cerebellar hemispheres, the superior cerebellar vermis, and parieto-occipital lobes, as indicated by the red arrows.
Mentions: She was subsequently noted to have a slowly rising Ca19-9 level, but without any symptoms or evidence of systemic disease progression on repeat radiographic imaging. After 12 months from initial diagnosis, the patient was brought to the emergency department with sudden onset of headaches, slurred speech, generalized weakness, and agitation. At this point, the Ca19-9 level was 1920 U/mL. Initial CT of the head with and without contrast showed evidence of leptomeningeal enhancement involving the posterior fossa (Figure 1). Analysis of cerebrospinal fluid (CSF) obtained via lumbar puncture revealed mildly elevated protein level of 50 mg/dL (reference range = 15-45 mg/dL), with normal glucose level of 67 mg/dL (reference range = 40-70 mg/dL). Cytological examination of CSF demonstrated malignant cells that were morphologically and immunohistochemically identical to the original biopsy from the periaortic lymph node metastasis (Figure 2). Magnetic resonance imaging of the brain and spine was consistent with abnormal leptomeningeal enhancement involving bilateral cerebellar hemispheres, vermis, parieto-occipital lobes, as well as diffuse enhancement of the spine (Figures 3 and 4). Treatment with intrathecal chemotherapy was discussed; however the family opted for palliative options and the patient rapidly deteriorated and expired.

Bottom Line: A 45-year-old woman was found with mediastinal and abdominal lymphadenopathy with lymph node biopsy consistent with adenocarcinoma, expressing immunomarkers CK7, CK20, and Ca19-9 along with markedly elevated serum Ca19-9 level.She was then noted to have slowly rising Ca19-9 level that did not correlate with her lack of evidence of systemic disease progression.Eventually, she presented with neurologic symptoms and was found on imaging to have isolated LMD.

View Article: PubMed Central - PubMed

Affiliation: Hofstra North Shore-Long Island Jewish School of Medicine, New Hyde Park, NY, USA.

ABSTRACT
Leptomeningeal disease (LMD) can occur in a small percentage of patients with active metastatic cancer. However, we report a case of LMD occurring during disease remission in a patient with carcinoma of unknown primary with panreaticobiliary features. A 45-year-old woman was found with mediastinal and abdominal lymphadenopathy with lymph node biopsy consistent with adenocarcinoma, expressing immunomarkers CK7, CK20, and Ca19-9 along with markedly elevated serum Ca19-9 level. The patient was started on a pancreatic cancer directed chemotherapy regimen of Folfirinox (5-fluorouracil, leucovorin, oxaliplatin, irinotecan) and achieved complete response. She was then noted to have slowly rising Ca19-9 level that did not correlate with her lack of evidence of systemic disease progression. Eventually, she presented with neurologic symptoms and was found on imaging to have isolated LMD.

No MeSH data available.


Related in: MedlinePlus