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Patient had a diagnosis of essential thrombocythemia [9962/3] in 2002 and was treated with Hydroxyurea. In 2010, the patient was admitted with severe bone pain and a diagnosis described as, "The overall features of the biopsy are consistent with a fibrotic stage of a chronic myeloproliferative disorder. The presence of up to 15% CD34+ immature cells seen in the biopsy suggests that the patient is transforming to an acute myeloid leukemia." In addition, cytogenetic studies and molecular testing for JAK2 were ordered. These findings confirmed a myeloproliferative disorder. JAK2 mutation was not detected. The patient died within 2 weeks. Is this a new primary?

Was this patient diagnosed with AML (which requires 20% or more blasts and this is only 15%)? If this is a new primary, is the histology 9861/3 [AML, NOS] or 9895/3 [AML with myelodysplasia-related changes]? Was the second diagnosis of AML definitively diagnosed?

Facility A: 4/8/2010 Bone Marrow biopsy: Features most compatible with MDS. (No treatment administered.) 7/2/2010 Peripherial Blood: Transforming Myelodysplastic Syndrome (MDS). COMMENT: Clonal abnormality compatible with MDS/acute myeloid leukemia (AML) in all metaphases examined. (Still no treatment administered.)

Facility B: 10/6/2010 Patient now presents for evaluation and treatment. Patient started on Vidaza. 10/07/10 Bone Marrow biopsy: Refractory anemia with excess blasts (RAEB-2) COMMENT: Evolution towards AML with myelodysplasia related changes considered; cytogenetic analysis reveals abnormalities most compatible with MDS and/or AML.

Based on the Heme Manual and DB, the 4/8/2010 diagnosis of MDS, NOS (9989/3) is the first primary. Should the 7/2/2010 diagnosis of transforming MDS to AML (9861/3) be a new, second primary?

Based on the Abstractor Note for MDS in the Heme DB for MDS, "If the characteristics of a specific subtype of MDS develop later in the course of the disease, change the histology code to the more specific diagnosis." Based on this note, should the MDS histology code [9989/3] be changed to refractory anemia with excess blasts (RAEB-2) [9983/3] from the biopsy taken on 10/7/2010 (one day after treatment began) that revealed RAEB-2 with evolution towards AML?

4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored.

4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH.

5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia.

6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma.

Patient presented with hoarseness and palpable neck mass. No palpable adenopathy (per hospital abstract).

02/19/16 Thyroid Ultrasound: Right thyroid lobe with mass, 63X35X44XMM (per hospital abstract).

06/01/16 Right thyroid lobectomy, radical resection right laryngeal tumor (per hospital abstract).

06/01/16 Operative Procedure: Tumor was invading laryngeal soft tissue and cartilage anteriorly and to the right. There may be a small amount of residual tumor invading cartilage although this was not clear (per hospital abstract).

GROSS DESCRIPTION: 1. The specimen is received fresh for intraoperative consultation, labeled with the patient's name and "right thyroid mass." It consists of a 3.0 x 2.2 x 2.0 cm irregular, ragged fragment of tan-red, firm, rubbery soft tissue. The specimen is serially sectioned to reveal a tan-red, gritty cut surface with focal fleshy areas. A touch prep is performed. A representative section is submitted for frozen section analysis in 1FSA. A portion of tissue is submitted for flow cytometry with the accession number MSO-16-1786. The remaining specimen is entirely submitted in 4 additional cassettes (1B-1E). 2. The specimen is received in formalin and is labeled "right thyroid lobe." It consists of a thyroid lobe measuring 4.3 x 4.0 x 1.3 cm and weighing 10.0 g. The external surface is covered by a thin fibrous capsule with a focal area of roughening on the posterior surface. The lobe is inked black posterior, blue anterior and orange isthmus margin. Serial sectioning reveals a red-brown and beefy parenchyma. A definitive nodule is not grossly identified. The entire specimen is serially submitted from superior to inferior in 9 cassettes. 3. The specimen is received in formalin, labeled with the patient's name and "right neck/laryngeal mass." It consists of an irregular, focally nodular red-tan mass measuring 7.0 x 5.5 x 4.0 cm and weighing 54 g. The convex portion of the specimen is mostly encapsulated with focal adherent red-brown striated skeletal muscle. The concave portion of the specimen is focally ragged and disrupted. The convex portion of the specimen is inked black and the concave portion is inked blue. The specimen is serially sectioned to reveal a white-grey to red, granular, gritty cut surface with focal fleshy areas. Representative sections are submitted in 12 cassettes.

Final DX DIAGNOSIS: 1. Right thyroid mass excision Plasma cell tumor /plasmacytoma 3 cm. Tumor cells are positive for kappa and negative for lambda immunostains. Recommend correlation with flow cytometry MSO-16-1786, monoclonal plasma cell population with cytoplasmic kappa positivity. Ki-67 stains 7 percent of cells. Focal stromal hyalinization. Congo red stain for amyloid negative. No thyroidal tissue identified. 2. Right thyroid lobe excision Benign thyroid tissue with focal solid cell nest negative for malignancy. One out of two 1/2 perithyroidal lymph nodes positive for plasma cell tumor. 3. Laryngeal mass excision Plasma cell tumor /plasmacytoma 7 cm involving soft tissue and skeletal muscle. Tumor cells are positive for kappa and negative for lambda immunostains. Ki-67 stains 7 percent of cells. Focal stromal hyalinization and calcification. Congo red stain for amyloid negative

Patient had a hemicolectomy and a salpingo-oophorectomy and was found to have diffuse large B cell lymphoma in the colon (10 cm cecal mass), 3/16 regional lymph nodes involved with lymphoma. Fallopian tube showed involvement with diffuse large B Cell lymphoma.

Multiple primaries - Colon and fallopian tube?

One primary - Colon? Stage IV, or lymphoma from an unknown primary? Note: There were no other lymph nodes involved.

Are peritoneal implants mets and not counted as separate tumors, even though they're not stated to be metastatic in the path report, and are not coded as distant mets?

8/1/15 CT Exam showed enlarged prostate and left seminal vesicle with multiple enlarged pelvic LNs. Findings: suspicious for prostate cancer with invasion of seminal vesicle. Bone scan findings: positive bone mets in multiple sites. PSA 169.0 (elevated). Patient was started on casodex 8/12/15. A prostate biopsy was performed on 9/16/15 to confirm diagnosis, adenocarcinoma Gleason 4+5. Patient's treatment continued with radiation to bone.

Aspirin is listed as treatment for "thrombocythemia" in the Abstracting and Coding Guide for the Hematopoietic Diseases but not for "primary polycythemia."