Heme Database Exercise Use the Hematopoietic Database to answer the following questions

Transcription

1 Heme Database Exercise Use the Hematopoietic Database to answer the following questions 1. Assign a topography and histology code to polycythemia vera 2. List two alternate names for refractory anemia 3. List two reportable diseases that Waldenstrom macroglobulinemia can transform to 4. List at least one definitive diagnostic method for Sezary s Disease. 5. What grade code should be assigned to mantle cell lymphoma? 6. Assign a histology for a Hodgkin lymphoma. 7. An extraosseous plasmacytoma may transform into 8. The corresponding ICD 9 code for Mantle Cell Lymphoma is 9. List two histology codes that would be considered the same primary as myelodysplastic syndrome 10. List two treatments for polycythemia vera

2 Lymphoma Case Scenario 1 A 31 year old black female presents with a history of autoimmune hemolytic anemia that has been treated with prednisone and rituxan. She has been referred here for a splenectomy. 4/18/13 CT Abd/Pelvis: Lung bases are clear. Liver, pancreas, both adrenals and both kidneys are within normal limits. Gallbladder is grossly within normal limits. Spleen is normal in size and demonstrates homogeneous enhancement. Small accessory spleen anterior and superior to the splenic hilum seen. There are a few prominent aortocaval lymph nodes in the retroperitoneum with the largest seen on image 27 measuring to about 10 mm in short axis. These are likely reactive lymph nodes though can be evaluated on a followup study if needed. A few prominent lymph nodes in the region of the porta hepatis and near the head of the pancreas also noted. These can also be reevaluated on followup exam. Bowel is not obstructed. Appendix is visualized and has normal configuration. Uterus and ovaries are grossly normal in appearance. A few pelvic phleboliths are seen. No pelvic or inguinal lymphadenopathy seen. SUMMARY: 1) Spleen is normal in size and enhancement pattern. 2) A few prominent lymph nodes in the retroperitoneum and in the peripancreatic region which are likely reactive but can be reevaluated on 3 to 4 month followup exam. 5/1/13 Splenectomy: Final Diagnosis: A) ACCESSORY SPLEEN, EXCISION: - NEGATIVE FOR MALIGNANCY. B) SPLEEN, SPLENECTOMY: - CLASSICAL HODGKIN LYMPHOMA, NODULAR SCLEROSIS TYPE. SPLENIC HILAR LYMPH NODE: - CLASSICAL HODGKIN LYMPHOMA, NODULAR SCLEROSIS TYPE. 5/10/13 Bone Marrow Bx: NORMOCELLULAR MARROW WITH TRILINEAGE HEMATOPOIESIS. - NO EVIDENCE OF MARROW INVOLVEMENT BY HODGKIN LYMPHOMA. 5/14/13 CT C/A/P: CT chest: No evidence of significant axillary lymphadenopathy. There has however been interval development of significant right paratracheal mediastinal lymphadenopathy causing shift of the trachea to the left. The largest right paratracheal nodes have maximum transverse diameter of 3.5 x 2.8 CM and 3.8 x 3.8 CM. Right paratracheal lymph node mass extends for approximately 7.7 CM from cephalad to caudad. There is also evidence of right hilar adenopathy with a right hilar node measuring 3.3 x 2.9 CM. There is no left hilar adenopathy. No other mediastinal lymphadenopathy. Thoracic aortic arch and proximal great vessels are unremarkable. Cardiac chambers are at the upper limits of normal for size. No evidence of pericardial thickening or pericardial effusion. No significant hiatal hernia. Lung parenchymal

3 images demonstrate no findings of emphysema no bronchiectasis. Minor atelectatic changes are noted in the lung bases with tiny left pleural effusion. Lungs otherwise clear. CT abdomen: There is evidence of free intraperitoneal air consistent with history of recent splenectomy. A drainage catheter is noted in the left upper quadrant in the splenic bed. There is no significant abnormal residual fluid collection in the splenic bed. The liver is normal in size. No focal intrahepatic lesions are demonstrated. Gallbladder is normal in appearance. No evidence of cholelithiasis or biliary duct dilatation. There are some inflammatory changes adjacent to the tail of the pancreas. This may be postoperative change related to the splenectomy although mild pancreatitis could have a similar appearance and correlation with serum amylase/lipase levels would be recommended. The pancreatic body and head are otherwise unremarkable. There is no significant peripancreatic fluid nor ascites. No adrenal lesions are demonstrated. No focal renal mass lesions are demonstrated. No evidence of urolithiasis or hydronephrosis. No evidence of abscess or ascites. Nonspecific slightly prominent itoneal lymph nodes are demonstrated with maximum transverse diameter of 1.3 CM just below the level of the renal hila. No other evidence of adenopathy. The bowel gas pattern suggests ileus with mild gaseous distention of colon and small bowel. No evidence to suggest mechanical bowel obstruction abdominal aorta and IVC are unremarkable. CT pelvis: The uterus and adnexa are unremarkable. No pelvic mass or adenopathy. No inflammatory change, abscess or ascites. Air is present in the urinary bladder. This is most commonly the result of recent catheterization although if there has not been a recent catheterization bowel to bladder fistula would have to be considered. No evidence of inguinal adenopathy. IMPRESSION: 1. Interval development of extensive right paratracheal mediastinal lymphadenopathy as well as hilar lymphadenopathy. 2. Slightly prominent retroperitoneal lymph nodes are also present, the largest in the aortocaval region just below level of the renal hila measuring up to 1.3 cm in diameter. No other evidence of adenopathy. 3. Postoperative changes of splenomegaly left upper quadrant with residual free intraperitoneal air. 4. Inflammatory changes surrounding the tail of the pancreas. This may represent postoperative change related to splenectomy although pancreatic tail pancreatitis could have a similar appearance and clinical correlation would be recommended. No other evidence of inflammatory change, abscess or ascites. 5/16/13 PET/CT: There are mildly enlarged, FDG avid lymph nodes at the base of the neck on the left. The maximal standard uptake value is in the range of 5.2. Similar appearing mildly enlarged lymph nodes with low level FDG uptake are seen within the prevascular space. More pronounced adenopathy/fdg uptake is seen within lymphoid tissue of the right supraclavicular fossa, extending to the base of the neck on the right and into the mediastinum on the right. Maximal SUV is in the range of There is extension along the right paratracheal space to the precarinal space and additional separate adenopathic uptake within the right hilum. Right hilar uptake is noted in the

4 range of There is no abnormal left hilar activity. No abnormal activity is present within the pulmonary parenchyma. There is a prominent lymph node along the ventral left lateral aspect of the distal esophagus. This is seen on series 2 image 8 of the comparison computed tomography study. This is FDG avid with a maximal SUV of 5.1. Subdiaphragmatically, there is activity within the left upper quadrant appearing related to recent splenectomy. There is prominent FDG activity related to conglomerate nodal disease within the region of the porta hepatis with a maximal SUV of There are also FDG avid lymph nodes within the proximal left paraaortic space and aortocaval space with maximal SUV of There is a solitary subcentimeter lymph node within the left inguinal region seen on series 2 image 86 which demonstrates some mild FDG uptake. The maximal SUV is 4.3. This is nonspecific and could be reactive in nature. There are regions of uptake within the abdominal wall on the left corresponding with recent splenectomy. There is also some linear stranding extending from the skin surface to the posterior aspect of the left iliac bone with mild FDG uptake which is likely due to some recent intervention. There is no abnormal lymph node activity seen within the gluteal regions. There is activity noted within bilateral adnexa. This appears most likely physiologic with no distinct adenopathy identified on the comparison computed tomography exam. The liver, and adrenal glands appear normal. There is normal urinary tract excretion. There is no abnormal focal marrow uptake. There is some mild symmetric cutaneous activity within the axilla. This is nonspecific, however given the symmetric appearance this is most likely benign. IMPRESSION: 1. Adenopathic uptake within the right supraclavicular fossa, involving the base of the neck bilaterally, with extension into the mediastinum and involvement of the right hilum. There is also adenopathic uptake within the inferior aspect of the mediastinum along the ventral aspect of the distal esophagus as well as within the proximal abdominal retroperitoneum and within the porta hepatis region. Findings are consistent with history of Hodgkin lymphoma. 2. Since the comparison computed tomography, there has been splenectomy. Activity within the left upper quadrant and within the abdominal wall is consistent with postsurgical changes. 3. Additional activity extending through the subcutaneous fat to the posterior left iliac bone likely due to recent intervention. 4. Physiologic appearing bilateral adnexal uptake and nonspecific but likely benign activity seen symmetrically within the cutaneous tissues of the axilla. 5. Low level nonspecific activity within non-enlarged solitary left inguinal lymph node. 5/21 Adriamycin, vinblastine, dacarbazine

5 Case Scenario 1 Worksheet Primary Site Morphology Grade Stage/ Prognostic Factors CS Tumor Size CS SSF CS Extension CS SSF CS Tumor Size/Ext Eval CS SSF CS Lymph Nodes CS SSF CS Lymph Nodes Eval CS SSF Regional Nodes Positive CS SSF Regional Nodes Examined CS SSF CS Mets at Dx CS SSF CS Mets Eval CS SSF CS SSF 1 CS SSF CS SSF 2 CS SSF CS SSF 3 CS SSF CS SSF 4 CS SSF CS SSF 5 CS SSF CS SSF 6 CS SSF CS SSF 7 CS SSF CS SSF 8 CS SSF Summary Stage Clinical AJCC TNM Stage Diagnostic Staging Procedure Surgery Codes Surgical Procedure of Primary Site Scope of Regional Lymph Node Surgery Surgical Procedure/ Other Site Systemic Therapy Codes Chemotherapy Hormone Therapy Immunotherapy Hematologic Transplant/Endocrine Procedure Systemic/Surgery Sequence Pathologic AJCC TNM Stage Treatment Radiation Codes Radiation Treatment Volume Regional Treatment Modality Regional Dose Boost Treatment Modality Boost Dose Number of Treatments to Volume Reason No Radiation Radiation/Surgery Sequence

6 Case Scenario 2 A 54 year old white male presents with a left inguinal mass that has been present for about a month. The patient denies weight loss, fever, chills, or drenching night sweats. 6/24/13 CT pelvis: There is adenopathy in the inferior retroperitoneum in the periaortic and pericaval regions. The common iliac arteries are tortuous. Moderate additional adenopathy is seen adjacent along the pelvic sidewalls bilaterally. The largest adenopathy is present in the left inguinal region. The bulging contour questioned on physical exam in the left inguinal region is due to the presence of several enlarged lymph nodes with the largest measuring 5 cm in diameter. Malignancy such as lymphoma to be excluded. Fat-containing inguinal hernias are present bilaterally. There is no pelvic ascites. Distal left colonic diverticulosis is noted but no evidence of diverticulitis. The appendix is normal. No intestinal dilatation. The left ureter is not opacify on the images obtained which may be related to the timing of the CT imaging. The distal left ureter is visualized and does not appear to be dilated. On bone window images there is mild lower lumbar facet joint arthropathy. No destructive bony lesions are seen. IMPRESSION: 1. Moderate amount of pelvic adenopathy including the inguinal regions. Multiple large left inguinal lymph nodes with the largest measuring 5 cm. Malignancy such as lymphoma to be excluded. Due to the amount of pelvic adenopathy, consider performing abdominal CT to further evaluate the extent of disease. 2. Left colonic diverticulosis. 7/6/13 Left inguinal LN excision: Follicular lymphoma, WHO gr 1 7/18/13 LD: 143 (Range ) 7/22/13 Bone Marrow Bx: NORMOCELLULAR MARROW WITH TRILINEAGE HEMATOPOIESIS. - NO EVIDENCE OF MARROW INVOLVEMENT BY LYMPHOMA. 7/25/13 CT C/A/P: CT CHEST: The heart is within normal limits in size. No pericardial effusion or pericardial thickening is seen. The ascending and descending thoracic aorta, as well as, the main, right and left pulmonary arteries are patent and within normal limits in caliber. The trachea and main stem bronchi demonstrate no intrinsic lesions or extrinsic compression. There is right axillary adenopathy which measures up to 1.2 x 2.4 cm. There is a lymph node anterior to the medial aspect of the right main stem bronchus measuring up to 1.6 cm. There is a calcified subcarinal lymph node measuring 1.5 x 2.6 cm. The esophagus is within normal limits in appearance. The lungs are well expanded without focal infiltrates. No pleural effusions or pneumothorax seen, bilaterally. CT ABDOMEN: The liver, spleen, pancreas and adrenal glands are unremarkable. The gallbladder is unremarkable. No evidence of intrahepatic or extrahepatic biliary duct dilatation is seen. The kidneys are within normal limits in shape, size and position without evidence of hydronephrosis or hydroureter, bilaterally. The kidneys are symmetrically perfused and excretory function is preserved. No focal renal

7 mass is seen. The abdominal aorta and inferior vena cava are patent and within normal limits in caliber. The stomach is unremarkable. Visualized segments of small and large bowel are within normal limits in caliber and appearance. The appendix is within normal limits in appearance. There are multiple enlarged retroperitoneal lymph nodes measuring up to 1.7 cm. CT PELVIS: Scattered colonic diverticula are present without imaging findings to suggest acute diverticulitis. The urinary bladder is well distended and is otherwise unremarkable. The prostate gland is not enlarged. No ascites or pneumoperitoneum is seen. There is extensive pelvic adenopathy which is not significant changed from the previous examination. There to adjacent enlarged left inguinal lymph nodes measuring 5.1 cm and 3.1 cm, respectively. There bilateral external iliac lymph nodes measuring up to 2.5 centers on the right and 2.7 cm on the left which is not significantly changed. IMPRESSION: 1. Extensive pelvic adenopathy which is unchanged. Thoracic and retroperitoneal adenopathy as detailed above. 2. No acute cardiopulmonary disease. 3. No evidence of a bowel obstruction or acute appendicitis. 4. Diverticulosis coli without acute diverticulitis. 5. No evidence of acute obstructive uropathy. First cycle of fludarabine, mitoxantrone and Rituxan will start on August 22, 2013.

8 Case Scenario 2 Worksheet Primary Site Morphology Grade Stage/ Prognostic Factors CS Tumor Size CS SSF CS Extension CS SSF CS Tumor Size/Ext Eval CS SSF CS Lymph Nodes CS SSF CS Lymph Nodes Eval CS SSF Regional Nodes Positive CS SSF Regional Nodes Examined CS SSF CS Mets at Dx CS SSF CS Mets Eval CS SSF CS SSF 1 CS SSF CS SSF 2 CS SSF CS SSF 3 CS SSF CS SSF 4 CS SSF CS SSF 5 CS SSF CS SSF 6 CS SSF CS SSF 7 CS SSF CS SSF 8 CS SSF Summary Stage Clinical AJCC TNM Stage Diagnostic Staging Procedure Surgery Codes Surgical Procedure of Primary Site Scope of Regional Lymph Node Surgery Surgical Procedure/ Other Site Systemic Therapy Codes Chemotherapy Hormone Therapy Immunotherapy Hematologic Transplant/Endocrine Procedure Systemic/Surgery Sequence Pathologic AJCC TNM Stage Treatment Radiation Codes Radiation Treatment Volume Regional Treatment Modality Regional Dose Boost Treatment Modality Boost Dose Number of Treatments to Volume Reason No Radiation Radiation/Surgery Sequence