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The patient was diagnosed with multiple myeloma, underwent treatment and subsequently was in remission. The patient later presented with lesions on the scalp and thorax lesions. The final diagnosis on the pathology report for the scalp lesion was multiple myeloma with plasmablastic transformation (high grade). The physician states this is an aggressive, recurrent multiple myeloma with multiple large plasmacytomas involving the scalp and thorax.

The patient was diagnosed with intervertebral disc plasmacytoma and had radiation therapy to the pelvic bones in 2010. In 2011 (more than 21 days later) a right hip biopsy revealed immature plasma cell myeloma. There is clinical documentation that this is progression into myeloma. Per the Heme DB (Primary Site(s) and Definition sections) and Rule PH30, in the Heme Manual, the primary site is coded to C421 [bone marrow] and the histology is coded 9732/3 [plasma cell myeloma] when there is a clinical diagnosis of multiple myeloma and/or there is no documentation of a bone marrow biopsy or the results are unknown. This patient did have a bone marrow biopsy that indicates there are an increased plasma cells present; plasma cells represent less than 10%. The skeletal survey and bone scan did not reveal any further lesions.

Is this progression of disease because there is only one lesion in the right hip 8 months after the diagnosis of plasmacytoma? Or is this a second primary based on the right hip biopsy that showed plasma cell myeloma and the physician's documentation of disease progression? Plasmacytomas are usually single lesions. Would this disease process have multiple lesions if they are diagnosed at different times?

The patient was diagnosed with follicular lymphoma in the breast in 2008. Per notes, there was no evidence of disease again until 2011 when the patient presented with ocular lymphoma. The physician stated this was part of the same disease process as the prior breast diagnosis. The bone marrow was not involved in either case.

Is this a single primary (recurrence) of follicular lymphoma? Or are these multiple primaries because they arise in different extralymphatic sites?

The patient was diagnosed with essential thrombocythemia in 2007 and was treated with Hydrea. The 2009 bone marrow biopsy showed primary myelofibrosis which the physician states is a transition from the essential thrombocythemia. The Heme DB calls this two primaries.

The patient was diagnosed with chronic myelogenous leukemia in 2008 followed by a diagnosis of chronic lymphocytic leukemia in 2011. Per the physician statement, the patient started nilotinib in 10/2011 for CML.

The SEER*Rx Database lists CML and GIST as the only primary site/histology combinations treated using nilotinib. Can nilotinib also be coded as treatment for the CLL primary?

The patient was diagnosed with an extranodal DLBCL on a biopsy of the stomach. A bone marrow biopsy performed 16 days later showed no DLBCL, but demonstrated an abnormal CD5-positive B-cell population that was subsequently referred to as CLL/SLL by the physician. The peripheral blood was negative and showed only moderate thrombocytopenia.

Does rule M10 apply in this case? Abstract the acute neoplasm as a single primary (DLBCL) as there was only one pathology specimen (stomach biopsy) proving DLBCL and the bone marrow did not definitively identify CLL/SLL.

The patient was diagnosed with an EBV-positive plasmablastic lymphoma involving the left testis on radical orchiectomy in April 2019.

In September 2019, a plasmacytoma was found on a right mandibular mass biopsy. Imaging at that time revealed diffuse disease involving the thoracic spine and sinus involvement. The patient then underwent a resection of the T8 spinal/epidural tumor that also proved plasmacytoma.

Subsequently, the right mandibular mass and testis slides were reviewed (at an outside facility) and both were stated to be, The T8/epidural tumor pathology was not reviewed, so it is unclear if this is also assumed to be the same disease process as the right mandibular mass or still a separate, solitary plasmacytoma.

Additionally, some chart notes indicate the patient has plasmablastic lymphoma with a secondary diagnosis of plasmacytoma, while other chart notes state this is stage IV plasmablastic lymphoma involving all documented sites. Although the plasmablastic lymphoma and at least the plasmacytoma of T8 have different ICD-O-3 histology codes, the physicians do seem to be treating this as a single disease process.

The patient was diagnosed with a composite lymphoma of a cervical lymph node 8 years after diagnosis of follicular lymphoma that involved lymph nodes and organs on both sides of the diaphragm. The patient's follicular lymphoma was diagnosed in 2002.

In 2010 an excisional biopsy of a left neck lymph node showed classical Hodgkin lymphoma, nodular sclerosis type, grade 2 (predominant component) associated with (minor component) low grade follicular lymphoma (composite lymphoma).

Should the primary site for the 2010 primary be coded to C770 [lymph nodes of head, face & neck] or C778 [multiple lymph node regions]?

The patient was diagnosed with a carcinoid tumor of the small intestine. The pre-surgical work-up also identified a lung nodule that showed no octreotide uptake, but was indeterminate on biopsy. The imaging differential diagnosis included carcinoid, hamartoma, or a non-calcified granuloma. The patient underwent a resection of the primary small bowel tumor, and the physician noted the lung nodule was of unclear diagnosis. The physician stated a solitary lung metastasis would be atypical, but that lung metastatic involvement could not be ruled out. The physician recommended resection of the lung nodule to ensure that the patient was disease free. The lung wedge resection proved a pulmonary hamartoma.

The rules for coding Surgical Procedure of Other Site are not entirely clear. The definitions for First Course of Therapy in the SEER Manual do state that treatment includes, "Procedures that destroy or modify primary (primary site) or secondary (metastatic) cancer tissue." This would seem to exclude the lung resection as it did not destroy, modify or remove metastatic cancer tissue. However, the instructions for coding Surgical Procedure of Other Site do not address removal of distant sites that are not incidental. The lung resection was not incidental; the physician recommended it to ensure the lung was not involved, but it also disproved metastatic involvement. Should the Surgical Procedure of Other Site field be coded 0 (none) or 4 (non-primary surgical procedure to distant site) in this case?

The patient was diagnosed with a biopsy-proven 12/2020 LUL SCC treated with radiation only, followed by a right hilar lymph node biopsy in 07/2022, that proved “metastatic pulmonary adenocarcinoma” per pathology and treated with radiation, followed by a biopsy-proven 12/2022 RLL SCC to be treated with immunotherapy only. The imaging never definitively identified a lung tumor that can be assumed to be a primary adenocarcinoma tumor.  In 06/2022, a PET scan only described a “strongly PET positive Rt inferior hilar LN vs infrahilar pulmonary mass,” as well as the subsequently biopsy-proven SCC in the RLL (12/2022 SCC primary). The biopsy path indicates this was a right hilar lymph node metastasis and does not indicate this is an infrahilar pulmonary mass. No other PET positive pulmonary lesions were seen at the time.

The oncologist’s assessment indicates the right hilar node was the only positive finding on the biopsy, and it was unclear if this right hilar node metastasis was from the left lung or if the primary was “not detectable.” The oncologist summarized this as a LUL lung lesion radiated for SCC, a right hilar lesion radiated for adenocarcinoma, and a RLL lung lesion on pathology found to be SCC.

Should the interval occurring metastatic adenocarcinoma be accessioned as a separate lung, NOS primary based on the histology difference? While the Solid Tumor Rules do not apply to metastasis, the oncologist did treat these three malignancies separately and does not indicate the hilar lymph node metastasis was felt to be from either SCC primary.