School of Medicine and Health Sciences Poster Presentations

Title

Aggressive Signet Ring Cell Gastric Adenocarcinoma in a young man

Document Type

Poster

Keywords

Signet cell carcinoma; pancytopenia; Signet cell and bone marrow infiltration

Publication Date

Spring 2017

Abstract

Introduction:

Gastric cancer is the fifth most commonly diagnosed cancer and the third leading cause of cancer related death worldwide. There are two types of gastric carcinoma: intestinal, characterized by cohesive tumor cells arranged in glandular formation, and diffuse, which is poorly cohesive with crescent-shaped eccentric nuclei. The diffuse type of gastric carcinoma, including signet cell carcinoma, is common in patients less than 50 years of age and is associated with advanced disease and poor prognosis. Diffuse type of carcinoma mostly found in patients without classic risk factors for gastric carcinoma such as untreated H. Pylori, or smoking history.

Signet cell carcinoma has a high rate of bone marrow metastasis, and can present with severe cytopenias. Once bone marrow metastasis is diagnosed, average life expectancy is 11 to 121 days. Though the global incidence of gastric carcinoma has declined since 1970, incidence of diffuse-type carcinoma has increased by ten-fold.

Case presentation:

A 26 year old previously healthy man presented with 3 days of pre-syncope, back pain and weakness, along with recurrent epistaxis, fever and chills. On exam he was tachycardic and febrile and appeared pale with dry mucous membranes.

Serum studies showed a hemoglobin of 4.0 g/dL and a platelet count of 96 x 103 cells/microliter (compared to 13.9 and 163,000, respectively, twenty days prior). INR was 1.48 and alkaline phosphatase 305 IU/L.

Peripheral smear showed myelopthisic disease with teardrop cells, schistocytes, polychromasia, and nucleated red blood cells. CT demonstrated mediastinal, bilateral hilar, abdominal and retroperitoneal lymphadenopathy. MRI of thoracolumbar spine showed no pathological fracture but increased heterogeneous bone marrow signals.

Bone marrow /lymph node biopsies showed atypical hyperchromatic cells with a signet ring like appearance, positive for CK20 positive, negative for CK7 and TTF-1, suggesting a gastrointestinal primary cancer. Upper endoscopy showed an infiltrating mass in the greater curvature of the stomach and biopsy confirmed gastric signet ring cell adenocarcinoma. The patient received 4 months of palliative chemotherapy with Oxaliplatin, Mitomycin, Doxorubicin, Capecitabine but unfortunately died.

Discussion/Conclusion:

This case highlights the importance of keeping a broad differential diagnosis in a patient with pancytopenia. Our patient was a healthy young man and therefore initial suspicion for malignancy was low. He was eventually diagnosed with signet ring cell gastric carcinoma with bone marrow infiltration. Because of the rising incidence of signet ring cell carcinoma and its atypical presentation, physicians must be more vigilant about diagnosing this malignancy to maximize early diagnosis.

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Creative Commons License
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Poster to be presented at GW Annual Research Days 2017.

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Aggressive Signet Ring Cell Gastric Adenocarcinoma in a young man

Introduction:

Gastric cancer is the fifth most commonly diagnosed cancer and the third leading cause of cancer related death worldwide. There are two types of gastric carcinoma: intestinal, characterized by cohesive tumor cells arranged in glandular formation, and diffuse, which is poorly cohesive with crescent-shaped eccentric nuclei. The diffuse type of gastric carcinoma, including signet cell carcinoma, is common in patients less than 50 years of age and is associated with advanced disease and poor prognosis. Diffuse type of carcinoma mostly found in patients without classic risk factors for gastric carcinoma such as untreated H. Pylori, or smoking history.

Signet cell carcinoma has a high rate of bone marrow metastasis, and can present with severe cytopenias. Once bone marrow metastasis is diagnosed, average life expectancy is 11 to 121 days. Though the global incidence of gastric carcinoma has declined since 1970, incidence of diffuse-type carcinoma has increased by ten-fold.

Case presentation:

A 26 year old previously healthy man presented with 3 days of pre-syncope, back pain and weakness, along with recurrent epistaxis, fever and chills. On exam he was tachycardic and febrile and appeared pale with dry mucous membranes.

Serum studies showed a hemoglobin of 4.0 g/dL and a platelet count of 96 x 103 cells/microliter (compared to 13.9 and 163,000, respectively, twenty days prior). INR was 1.48 and alkaline phosphatase 305 IU/L.

Peripheral smear showed myelopthisic disease with teardrop cells, schistocytes, polychromasia, and nucleated red blood cells. CT demonstrated mediastinal, bilateral hilar, abdominal and retroperitoneal lymphadenopathy. MRI of thoracolumbar spine showed no pathological fracture but increased heterogeneous bone marrow signals.

Bone marrow /lymph node biopsies showed atypical hyperchromatic cells with a signet ring like appearance, positive for CK20 positive, negative for CK7 and TTF-1, suggesting a gastrointestinal primary cancer. Upper endoscopy showed an infiltrating mass in the greater curvature of the stomach and biopsy confirmed gastric signet ring cell adenocarcinoma. The patient received 4 months of palliative chemotherapy with Oxaliplatin, Mitomycin, Doxorubicin, Capecitabine but unfortunately died.

Discussion/Conclusion:

This case highlights the importance of keeping a broad differential diagnosis in a patient with pancytopenia. Our patient was a healthy young man and therefore initial suspicion for malignancy was low. He was eventually diagnosed with signet ring cell gastric carcinoma with bone marrow infiltration. Because of the rising incidence of signet ring cell carcinoma and its atypical presentation, physicians must be more vigilant about diagnosing this malignancy to maximize early diagnosis.