Small Bowel Obstruction

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  • Small Bowel Obstruction

    1. 1. An unusual cause of small bowel obstruction… Senior Clinicopathologic Conference Andrew S Kim, MD April 23, 2008 Walter Reed Army Medical Center
    2. 2. History of Present Illness <ul><li>49 y/o white male </li></ul><ul><li>Local to Landstuhl Hospital </li></ul><ul><li>Presents to the ED with c/o acute onset N/V and worsening abdominal pain </li></ul><ul><li>Intermittent abd pain x 1 week </li></ul><ul><li>ROS: Nausea, (+) bilious vomiting, abdominal distension </li></ul>
    3. 3. History of Present Illness <ul><li>Recent trauma “pinned” between car and a tree 4-5 months prior </li></ul><ul><li>Diagnosis of CMML-2 at Landstuhl </li></ul><ul><ul><li>Discovered during workup of thrombocytopenia for hernia </li></ul></ul><ul><ul><li>Diagnosis confirmed by both AFIP and Dana-Farber Cancer Institute </li></ul></ul>
    4. 4. Other Past Medical/Surgical History <ul><li>Benign Prostatic Hypertrophy </li></ul><ul><li>Right ACL Repair ‘06 </li></ul><ul><li>Right Inguinal Hernia repair ‘05 </li></ul><ul><li>NKDA </li></ul><ul><li>Not currently taking any medications </li></ul>
    5. 5. Social History <ul><li>Lifetime nonsmoker, nondrinker </li></ul><ul><li>Retired Air Force LTC </li></ul><ul><li>Remote travel hx </li></ul>
    6. 6. Physical Exam <ul><li>Vitals: afebrile bp 130/80 hr 98 rr 20 Pox 94% RA </li></ul><ul><li>GEN: moderate distress, WDWN, physically fit WM </li></ul><ul><li>CV: normal, no MRG </li></ul><ul><li>Pulm: clear to auscultation bilaterally </li></ul><ul><li>Ab: marked abdominal distension with midline abdominal fullness, mild tenderness to palpation, decreased bowel sounds throughout </li></ul><ul><li>Lymph: no abnormalities noted </li></ul>
    7. 7. Labs/Path Reports <ul><li>Previous Labwork (11/06) </li></ul><ul><ul><li>4.9 >12.7/38<92 </li></ul></ul><ul><ul><ul><li>50S/ 33M ; (AMC 1600) </li></ul></ul></ul><ul><ul><li>BMP normal </li></ul></ul><ul><ul><li>TP/Albumin normal </li></ul></ul><ul><ul><li>BCR-ABL (-) </li></ul></ul><ul><ul><li>t(5; 12) (-) </li></ul></ul><ul><li>ESR 6, CRP normal </li></ul><ul><li>AFIP/Dana-Farber report: CMML-2 </li></ul><ul><li>Admission Labwork (3/07) </li></ul><ul><ul><li>7>9.7/29.4<110 </li></ul></ul><ul><ul><ul><li>41S/54M (AMC 3600) </li></ul></ul></ul><ul><ul><li>140/3.7/106/26/25/0.9 </li></ul></ul><ul><ul><li>TP/Albumin 4.5/2.5 </li></ul></ul><ul><ul><li>LFTs normal </li></ul></ul><ul><ul><li>Coags normal </li></ul></ul>
    8. 8. Initial Hospital Course <ul><li>CT A/P: high grade obstruction in 3 rd part of the duodenum </li></ul><ul><li>Exploratory Laporotomy: </li></ul><ul><ul><li>Large inflammatory “tumor-like” mass along entire base of mesentery with significant encasement of small bowel </li></ul></ul><ul><ul><li>OR report describes mass as tan colored, studded “glue-like” consistency of lesion </li></ul></ul>
    9. 9. Initial Hospital Course <ul><li>Initial path report and frozen sections not representative of malignant cells but large “acute and chronic” inflammatory infiltrate </li></ul><ul><li>Intraoperative G- and J-tube placed </li></ul><ul><li>Transferred to WRAMC General surgery service </li></ul><ul><li>Continued intermittent, persistent obstructive sx </li></ul><ul><ul><li>GI, Heme-Onc consults </li></ul></ul>
    10. 10. Initial Hospital Course <ul><li>Transferred from Gen Sg to Medicine wards due to improvement in his sx </li></ul><ul><li>Treatment with high-dose steroids continued </li></ul><ul><li>Meanwhile, tissue results from his ex-lap came back as well as BM Bx and peripheral smear slides performed at LRMC </li></ul>
    11. 11. Tissue slides and BM biopsy: <ul><li>Dr. Jean Kemp </li></ul><ul><li>Department of Pathology </li></ul><ul><li>Walter Reed Army Medical Center </li></ul>
    12. 12. Mesenteric Panniculitis 2x
    13. 13. Mesenteric Panniculitis 20x
    14. 14. Mesenteric Panniculitis 40x
    15. 15. CMML 2x
    16. 16. CMML 10x
    17. 17. CMML 20x
    18. 18. CMML 40x
    19. 19. CMML 100x
    20. 20. Summary Biopsy Results: <ul><li>Mesenteric Panniculitis slides </li></ul><ul><ul><li>atypical histiocytic infiltrate with mixed inflammatory cells, consistent with mesenteric panniculitis (AFIP) </li></ul></ul><ul><li>CMML </li></ul><ul><ul><li>Diagnosis of CMML-1 vs CMML-2 </li></ul></ul><ul><ul><li>(Confirmed by Dana-Farber cancer institute) </li></ul></ul>
    21. 21. Mesenteric Panniculitis: Definition <ul><li>Synonyms: </li></ul><ul><ul><li>sclerosing mesenteritis </li></ul></ul><ul><ul><li>mesenteric lipodystrophy </li></ul></ul><ul><ul><li>lipogranuloma of the mesentery </li></ul></ul><ul><ul><li>sclerosing lipogranulomatosis </li></ul></ul><ul><ul><li>primary liposclerosis of the mesentery and multifocal subperitoneal sclerosis </li></ul></ul>
    22. 22. Mesenteric Panniculitis: Epidemiology <ul><li>Epidemiology: rare with no “true epidemiology” </li></ul><ul><ul><li>Prevalence~1% </li></ul></ul><ul><ul><ul><li>Age: 50-70s </li></ul></ul></ul><ul><ul><li>Male:Female 2:1 </li></ul></ul>
    23. 23. Mesenteric Panniculitis: Etiologies 8 <ul><li>Infection or ischemia </li></ul><ul><ul><li>Based on animal models, isolated case reports </li></ul></ul><ul><li>Autoimmune causes </li></ul><ul><ul><li>Thyroid disorders, AI pancreatitis, SLE, RPC </li></ul></ul><ul><li>Trauma/Surgery related </li></ul><ul><ul><li>AFIP series 8 </li></ul></ul><ul><li>Paraneoplastic causes </li></ul><ul><ul><li>Often the presenting complaint </li></ul></ul><ul><li>Largely only case reports and retrospective </li></ul><ul><li>Poorly defined pathophysiology </li></ul>
    24. 24. Mesenteric Panniculitis: Presentation <ul><li>Most common presenting sx 1-3 : </li></ul><ul><ul><li>Abdominal pain (70%) </li></ul></ul><ul><ul><li>Diarrhea (25%) </li></ul></ul><ul><ul><li>Wt loss (23%) </li></ul></ul><ul><ul><li>Small Bowel obstruction (24-36%) </li></ul></ul><ul><li>Physical Exam with LUQ or epigastric mass in up to 50% of pts 3 </li></ul><ul><li>Labs usually normal </li></ul>
    25. 25. Mesenteric Panniculitis: Diagnosis <ul><li>Differential Diagnosis includes all diseases of the mesentery </li></ul><ul><li>Combination of clinical hx and presentation, radiology and histology needed </li></ul>
    26. 26. Mesenteric Panniculitis: Radiology <ul><li>CT imaging : usually with findings of small bowel mesenteric inflammatory process </li></ul><ul><ul><li>“ fat ring” sign and “tumor pseudocapsule” somewhat specific 4,5 </li></ul></ul><ul><li>MRI emerging as a potentially useful tool 6 </li></ul><ul><ul><li>presence of fibrous capsule not seen in other disease processes </li></ul></ul><ul><li>PET scan? 7 </li></ul><ul><li>Mucosal preservation is a key difference between mesenteric panniculitis and other malignant processes </li></ul>
    27. 27. Mesenteric Panniculitis: Histology <ul><li>Most common findings: fibrous tissue, chronic inflammation </li></ul><ul><li>Flow cytometry may be utilized if lymphoma cannot be excluded on histologic evaluation </li></ul><ul><li>Immunohistochemistry may be useful in determining GIST and mesenteric fibromatosis from MP </li></ul>
    28. 28. Mesenteric Panniculitis: Prognosis <ul><li>Natural history not well understood </li></ul><ul><li>Generally felt to be benign course </li></ul><ul><li>Some trends noted in the literature 4-8 </li></ul><ul><ul><li>Idiopathic cases tend to have more favorable prognosis </li></ul></ul><ul><ul><li>Patients who develop intestinal obstruction have worse prognosis </li></ul></ul><ul><ul><li>Patients with underlying malignancy have the worst prognosis </li></ul></ul>
    29. 29. Mesenteric Panniculitis: Treatment 7-10 <ul><li>No universally recognized standard of care </li></ul><ul><li>MEDICAL THERAPY </li></ul><ul><ul><li>Steroids: most used in literature </li></ul></ul><ul><ul><li>Tamoxifen, Progesterone </li></ul></ul><ul><ul><li>Colchicine </li></ul></ul><ul><ul><li>Azathioprine </li></ul></ul><ul><ul><li>Thalidomide </li></ul></ul><ul><ul><li>Cytoxan </li></ul></ul><ul><li>OTHER THERAPIES </li></ul><ul><ul><li>Radiation therapy not useful </li></ul></ul><ul><ul><li>Surgical role limited </li></ul></ul>
    30. 30. Mesenteric Panniculitis: Cause? <ul><li>Trauma/Surgery related </li></ul><ul><li>Ischemia </li></ul><ul><li>Infectious causes </li></ul><ul><li>Autoimmune causes </li></ul><ul><li>Paraneoplastic causes </li></ul>
    31. 31. What’s left? <ul><li>Recent Trauma </li></ul><ul><li>Infectious </li></ul><ul><li>Autoimmune </li></ul><ul><li>Paraneoplastic </li></ul><ul><ul><li>Prior history of CMML-2 (more aggressive form of CMML) </li></ul></ul><ul><ul><li>No other malignant processes identified </li></ul></ul>
    32. 32. Chronic Myelomonocytic Leukemia: CMML <ul><li>Dr. Michael Marte/Dr. Andre Cap </li></ul><ul><li>Department of Hematology-Oncology </li></ul><ul><li>Walter Reed Army Medical Center </li></ul>
    33. 33. CMML - A Disease in the Shadows <ul><li>Clonal hematopoietic progenitor cell defect </li></ul><ul><li>Occupies the nexus between MDS and MPD </li></ul><ul><ul><li>Features of both, but consistent with neither </li></ul></ul><ul><li>Represents an epidemiologic difficulty </li></ul><ul><ul><li>Many grouped with CML or with MDS </li></ul></ul><ul><ul><ul><li>Accounts for as much as 13% of “MDS” </li></ul></ul></ul><ul><ul><li>Median age: 65-75 years </li></ul></ul><ul><ul><li>Male: Female ratio 1.5-3:1 </li></ul></ul>
    34. 34. The Faces of CMML <ul><li>Clinically either MDS-like, or MPD-like </li></ul><ul><ul><li>(MPD) - proliferative: dysplastic megakaryocytes </li></ul></ul><ul><ul><li>(MDS) - marrow failure: anemia, thrombocytopenia </li></ul></ul><ul><li>Diagnostic criteria: </li></ul><ul><ul><li>< 20% marrow blasts, < 5% peripheral blasts </li></ul></ul><ul><ul><ul><li>otherwise MDS (RAEB-1 vs. RAEB-2) </li></ul></ul></ul><ul><ul><li>Absolute peripheral monocyte count > 1,000/ μ L for 3 months </li></ul></ul><ul><ul><li>BCR/ABL negative </li></ul></ul><ul><ul><li>WBC count? </li></ul></ul><ul><ul><ul><li>If < 12,000/ μ L -> MDS; If > 12,000 -> MPD </li></ul></ul></ul><ul><ul><ul><li>Cases split roughly evenly </li></ul></ul></ul><ul><ul><li>Variable cytogenetics, essentially unhelpful </li></ul></ul>
    35. 35. The Faces of CMML <ul><li>Similar to RAEB-1, RAEB-2 classification </li></ul><ul><ul><li>CMML-1 </li></ul></ul><ul><ul><ul><li>Marrow blasts < 10% </li></ul></ul></ul><ul><ul><ul><li>Peripheral blasts < 5% </li></ul></ul></ul><ul><ul><li>CMML-2 </li></ul></ul><ul><ul><ul><li>Marrow blasts 10-19% </li></ul></ul></ul><ul><ul><ul><li>Peripheral blasts 5-19% </li></ul></ul></ul><ul><ul><ul><li>AUER rods and < 20% peripheral or marrow blasts </li></ul></ul></ul><ul><li>Progression to AML difficult to quantify, but not uncommon </li></ul>
    36. 36. Diagnosis of CMML <ul><li>Differential diagnosis </li></ul><ul><ul><li>CML </li></ul></ul><ul><ul><li>BCR/ABL-negative MPDs </li></ul></ul><ul><ul><li>MDS </li></ul></ul><ul><ul><li>Chronic eosinophilic/neutrophilic leukemia </li></ul></ul><ul><li>Helpful diagnostic strategies: </li></ul><ul><ul><li>BCR/ABL: most sensitive – PCR peripheral blood </li></ul></ul><ul><ul><li>Bone marrow biopsy with flow cytometry </li></ul></ul><ul><ul><ul><li>CD4, 14, 43, 56, 68 positive and characteristic </li></ul></ul></ul><ul><ul><li>Peripheral smear: Leukoerythroblastic appearance </li></ul></ul>
    37. 37. CMML - Presentation <ul><li>Fatigue, fever, night sweats, weight loss </li></ul><ul><li>Cytopenias </li></ul><ul><ul><li>Infection: relative neutropenia </li></ul></ul><ul><ul><li>Bleeding </li></ul></ul><ul><li>Extramedullary hematopoiesis/leukemic infiltration </li></ul><ul><ul><li>Spleen, liver, skin, lymph nodes, pulmonary parenchyma </li></ul></ul><ul><ul><li>Splenomegaly/hepatomegaly in proliferative disease </li></ul></ul>
    38. 38. CMML - Treatments <ul><li>Hyperproliferative -> cytoreduce </li></ul><ul><ul><li>Hydroxyurea is preferred agent </li></ul></ul><ul><ul><ul><li>Ara-C, etoposide, topotecan are second-line </li></ul></ul></ul><ul><li>Hypoproliferative </li></ul><ul><ul><li>Transfusions as needed </li></ul></ul><ul><ul><li>Growth factor support if: </li></ul></ul><ul><ul><ul><li>serum erythropoietiv < 500 mU/mL </li></ul></ul></ul><ul><ul><ul><li>Fewer than 2 units PRBCs transfused per month </li></ul></ul></ul><ul><ul><li>Hypomethylating agents </li></ul></ul><ul><ul><ul><li>Decitabine, Azacitidine </li></ul></ul></ul>
    39. 39. CMML and TKIs <ul><li>Myeloproliferative CMML </li></ul><ul><ul><li>Balanced translocations with resultant aberrant kinase activity </li></ul></ul><ul><ul><ul><li>TEL-PDGFR β receptor </li></ul></ul></ul><ul><ul><ul><ul><li>CMML-like disease in murine models </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Inhibited by SU11657 TKI (multi-kinase inhibitor) </li></ul></ul></ul></ul><ul><ul><ul><li>TEL-Jak2 t(5;12) </li></ul></ul></ul><ul><ul><ul><ul><li>Usually with pronounced eosinophilia </li></ul></ul></ul></ul><ul><ul><ul><ul><li>102% of CMML cases </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Responsive to Gleevec </li></ul></ul></ul></ul>
    40. 40. Transplant <ul><li>Represents the only curative option in CMML </li></ul><ul><ul><li>Preferentially reduce blast count prior to transplant </li></ul></ul><ul><li>Allogeneic transplant preferred </li></ul><ul><ul><li>GVL effect </li></ul></ul><ul><li>Minimal data with RIC transplantation </li></ul><ul><li>3Y DFS–39% (FHCRC), 5Y DFS– 18%(EBMT) </li></ul><ul><ul><li>TRM as high as 52% </li></ul></ul><ul><ul><li>Possibly improved in patients transplanted early </li></ul></ul>
    41. 41. <ul><li>Now back to our patient… </li></ul>
    42. 42. Subsequent Hospital Course <ul><li>Pt continued to require high dose steroids </li></ul><ul><li>Worsening malnutrition and persistent obstructive sx </li></ul><ul><li>MICU transfers (multiple) </li></ul><ul><ul><li>Upper GI bleed, multiple episodes </li></ul></ul><ul><ul><li>Gram negative sepsis requiring MICU care </li></ul></ul><ul><ul><ul><li>Lactate >10 </li></ul></ul></ul><ul><li>Daily high fevers to 103-104 continued throughout </li></ul>
    43. 43. Subsequent Hospital Course <ul><li>Discussions with patient and family regarding potential therapeutic options </li></ul><ul><ul><li>CTX versus bone marrow transplant </li></ul></ul><ul><li>Multiple conference calls CMML experts at the Mayo Clinic in Rochester, Minnesota </li></ul><ul><li>Transferred to Mayo after 8 weeks of inpatient care at Walter Reed Army Medical Center </li></ul>
    44. 44. The Mayo Clinic <ul><li>Initially offered “7+3” or decitabine for CMML-2 </li></ul><ul><ul><li>Received one course of Etoposide </li></ul></ul><ul><li>10 days into his hospital course there pt experienced an acute clinical decline with neutropenia, fever, and hemodynamic collapse and death </li></ul><ul><ul><li>Bone marrow biopsy repeated on transfer with evidence of hemophagocytosis </li></ul></ul>
    45. 45. The Mayo Clinic <ul><li>“ Hemophagocytic Syndrome” </li></ul><ul><li>( Hemophagocytic lymphohistiocytosis) </li></ul><ul><ul><li>Fever, HSMG, cytopenias and evidence of hemophagocytosis on bone marrow by macrophages, spleen or LN biopsy </li></ul></ul>www.healthsystem.virginia.edu/internet/hematology
    46. 46. Acknowledgements/Questions <ul><li>- Dr Marte/Dr Cap: WRAMC Heme-Onc </li></ul><ul><li>- Dr Kemp/Dr Schaffer: WRAMC Pathology </li></ul><ul><li>Dr Fadell/Labovich: LRMC Heme-Onc/Pathology </li></ul><ul><li>Dr Rosco Gore: WRAMC IM </li></ul>
    47. 47. References <ul><li>1. Khachaturian, T, Hughes, J. Mesenteric panniculitis. West J Med 1988; 148:700 </li></ul><ul><li>2. Durst, AL, Freund, H. Mesenteric Pannicluitis: review of the literature. Surgery 1977; 81: 203. </li></ul><ul><li>3. Akram, S, Pardi, DS, et al. Sclerosing mesenteritis: clinical features, treatment, and outcomes in ninety-two patients. Clin Gastro Hepatol 2007; 5: 589. </li></ul><ul><li>4. Horton, KM, Lawler, LP, et al. CT findings in sclerosing mesenteritis (panniculitis): Spectrum of disease. Radiographics 2003; 23: 1561. </li></ul><ul><li>5. Sabate, JM, Torrubia, et al. Sclerosing mesenteritis: imaging findings in seventeen patients. AJR Am J Roentgenol 1999; 172: 625. </li></ul><ul><li>6. Emory, TS, Monihan JM, Carr, NJ, et al. Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: A single entity? Am J Surg Pathol 1997; 21: 392. </li></ul><ul><li>7. Ogden, WW, Bradburn, DM. Mesenteric Panniculitis: Review of twenty-seven cases. Ann Surg 1965; 161: 184. </li></ul><ul><li>8. Bala, A, Coderre, SP et al. Treatment of sclerosing mesenteritis with corticosteroids and azathioprine. Can J Gastroent 2001; 15: 533. </li></ul><ul><li>9. Genereau, T, Bellin, MF, et al. Demonstration of efficacy of combining steroids and colchicine in two patients with idiopathic sclerosing mesenteritis. Dig Dis Sci 1996; 41: 684. </li></ul><ul><li>10. www.uptodateonline.com </li></ul>

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