|Case of the Week - Patient Summary 13428|
Peer Reviewed and Certified -
|Demographics: 50 y.o. woman|
|History & Chief complaint:|
| This 50-year-old post menopausal woman presented with progressive back pain of two months duration with worsening after a fall two weeks ago. She reports night sweats but no fever, chills or weight loss. She also reports some lower extremity weakness.
|Physical exam and Laboratory:|
| PE is remarkable only for focal back pain, there was no cervical or supraclavicular adenopathy. Patient was Afebrile with stable vital signs. CA-125 of 48 and LDH of 740 were both elevated. |
|Summary of Findings:|
|MRI showed L3 compression fracture and large pelvic mass. Subsequent CT with contrast revealed a 12 x18 heterogeneous multiloculated pelvic mass with adjacent collections of low density free fluid, a soft tissue density mass measuring 4 x 7.4 cm at superior pole of right kidney and a 7.6 x 7.2 cm fluid-filled collection anterior to ascending colon. An aortocaval lymphnode measuring 12x13 mm and additional sub-centimeter nodes were identified. Interval CT evaluation after second cycle of CHOP-R treatment showed complete resolution of Right renal mass, interval decrease of aortocaval node, and interval decrease of pelvic mass now measuring 12.7 x 7.2 cm. The L3 compression fracture is stable.|
|(1) Ovarian Carcinoma (2) Acute leukemia, 3) Hepatocellular carcinoma, 4) Non Hodgkin’s Lymphoma, (5) Pancreatic tumor|
| Diffuse Large B-cell Lymphoma |
|Confirmed by: Pathology of CT guided biopsy of pelvic mass and right peri-renal lymph showed this to be Diffuse Large B-cell lymphoma|
|Treatment and Followup:|
|Chemotherapy CHOP-Rituximab six cycles. Evaluation for adjuvant radiation thereapy may be warranted.|
|Patient Specific Discussion: (Also Read the Disease Discussion)|
|MRI of spine showed L3 compression fracture and incidental finding of a large pelvic mass. She reports night sweats but no fever, chills or weight loss. The mass was worrisome for malignancy. The patient also reports some low extremity weakness. The patient has an IPI (International Prognostic Index) of 3, meeting criteria for LDH, stage, and extra-nodal disease and thus appears to have a poor prognosis. In the presence of a pathological bone fracture (L3) the patient’s clinical condition raises the suspicion whether this may either be due to primary or secondary Non Hodgkin’s lymphoma of the bone. In a retrospective study of 23 patients with Primary Non Hodgkin’s lymphoma of the bone (PLB) it was found that most were due to Diffuse Large B-cell lymphoma and three cases involved pathologic bone fractures. According to this study Computed Tomography and roentgenography alone were not sufficient to diagnose PLB and that diagnosis must be confirmed according to clinical features (localized pain and tenderness), pathological findings and immunohistochemistry assay . Our patient meets these confirmatory measures and given the fact that the therapeutic procedure for PLB mainly includes local radiation therapy combined with chemotherapy, our patient may best benefit from adjunctive radiotherapy.
|Diffuse Large B-Cell Lymphoma (DLBCL) is the most common cause (30%) of Non Hodgkin's Lymphoma (NHL). DLBCL may be associated with B symptoms: fever, weight loss and drenching sweat. In 40% of cases the disease is extra-nodal and most commonly affects the stomach/gastro-intestinal tract but may also involve other sites - include the CNS.
DLBCL commonly affects the middle aged and the elderly and the average age is 64 yrs. It has a slightly higher incidence in men and 60 % of people are diagnosed at stage III/IV.
Treatment and prognosis depends on Ann Arbor staging (Stage I, II, III, and IV). Current standard treatment for DLBCL is CHOP-Rituximab given every three weeks for six to eight cycles. Survival of DLBCL is based on the International Prognostic Index IPI) and is decreased by presence of five parameters each with a point index of 1: age > 60 yrs, elevated LDH, performance status greater or equal to 2, Advanced Stage (III/IV), and number of involved extra nodal disease. A Revised International Prognostic Index predicts outcomes as follows: IPI score of 0 as Very good (94% overall survival, 94% progression free survival), IPI score of 1 or 2 as Good (79% overall survival, 80% progression free survival), and IPI score of greater or equal to 3 as poor (55% overall survival, 53% progression free survival).
There is evidence that the presence of elevated CA-125 appears to be a useful predictor of aggressive histology and bulky disease and its presence at the time of diagnosis has also been associated with a decreased 5-year survival. Data indicate that serum CA 125 is a useful tumor marker in NHL patients that may improve staging and CA 125 levels reflect the patient's response to the invasive potential of the tumor, giving a measurement of its infiltrative activity.
Recommended follow up is with CT scans at months 6, 12, 18, 24, 30 or 36 months and then yearly until five years after attainment of a complete remission. No more imaging is performed after five years unless an abnormality suggests the possibility of relapse or the patient requests it.
Following completion of treatment, some patients may have residual masses on CT scanning containing only fibroticc tissue or non-viable tumor and may be mistakenly considered as not having achieved complete remission.
PET scanning or combined PET/CT scanning is suggested to resolve most cases. Whenever PET-positive disease at the completion of treatment is identified a tissue biopsy or further evaluation is required, as some of these patients remain in prolonged remission. PET or PET/CT imaging is not used routinely in the longitudinal follow-up of asymptomatic patients after response assessment.