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Lutathera is a radioconjugate consisting of the tyrosine-containing somatostatin analog Tyr3-octreotate (TATE) conjugated with the bifunctional, macrocyclic chelating agent tetra-azacyclododecanetetra-acetic acid (DOTA) and radiolabeled with the beta-emitting radioisotope lutetium Lu 177 with potential antineoplastic activities.

Lymphoma case diagnosed in 2021: The patient had first round of systemic therapy as documented in the treatment plan and a post-chemotherapy PET scan that showed residual disease. The patient then had a different combination of systemic therapy and still had some residual disease. The patient was given a third round of different combination of systemic therapy in preparation for stem cell transplant. According to the physician post-stem cell transplant note, the patient achieved complete remission.

Is the first course of therapy the first round of systemic therapy only or is it all the therapy given to achieve remission?  It seems like only the first round of systemic therapy is first course of therapy for both leukemia and lymphoma in the hematopoietic manual. I thought all treatment for all hematopoietic cases was first course until remission achieved or progression was evident.

MCAS has been given an ICD-9 code of 202.60 by our medical record coders. In the Progress Notes, the physicians state this is not the same as systemic mastocytosis. There is no listing for MCAS in the Hematopoietic and Lymphoid Database.

MP/H rule M5 states that melanomas with ICD-O-3 histology codes that are different at the third number are multiple primaries. However, the 2007 MP/H fundamentals Webcast session on melanoma rules states that this is not two histologic types. Lentigo maligna is a growth pattern, not a histologic type. Will clarification be included in the next MP/H rules revision?

MP/H rules do not apply to metastasis. However, it has been our experience that pathologists and clinicians tend to use the terms metastatic and recurrence interchangeably. The term "recurrence" is not limited to a tumor recurrence in the same site as a previous malignancy. Sometimes it is obvious that the clinician is using the term recurrence to describe a metastatic lesion. When a "recurrence" is located in tissue that is very different from the original primary site, it is easy to recognize that the intended meaning of the term is metastasis.

Example: Patient with squamous cell carcinoma of the tongue with recurrence in the lung.

However, when the metastatic deposit occurs in similar tissue, it is more difficult to determine the number of primaries.

Example when the term "recurrence" is ambiguous: In April 2008 patient was diagnosed with adenocarcinoma of the ascending colon. At the time of hemicolectomy the tumor was noted to be plastered into the paraduodenal and peripancreatic area. Patient received one dose of adjuvant chemo and then discontinued treatment. In May 2009 the patient was found to have adenocarcinoma in the transverse colon. Per the pathology report the diagnosis for segmental resection at that time showed colonic adenocarcinoma. Tumor location: tumor appears recurrent at anastomotic junction. Abdominal wall mass showed metastatic adenocarcinoma.

One has to wonder if the pathologist found a metastatic nodule at the anastomotic site and called it "recurrent." It is unlikely that the pathologist will compare this specimen to the previous tumor, having already diagnosed it as "recurrent."

MRI Lumbar spine: Bony metastatic disease most evident at L5, L3 and T10. There is marrow tumor in the posterior elements of T12 and T10. The 14 mm epidural mass represents epidural tumor, likely metastatic, extending into the left intervertebral foramen at T12-L1.

PET scan: Hypermetabolic activity corresponding to epidural mass at the level of T12 and L1 concerning for malignancy. Other small areas of hypermetabolic activity in the left mandible and both femoral necks. There is no hypermetabolic activity corresponding to the areas of abnormal marrow edema in the vertebral bodies which enhanced on MRI scan in the lumbar and lower thoracic spine. No lymph nodes mentioned.

Biopsy epidural mass: Diffuse large B-cell lymphoma with a background of follicular lymphoma, consistent with a large cell transformation. Flow cytometry confirms a mixed large and small cell population of lymphoma (55% large cells).

T12/L1 Bone Biopsy: Bone and marrow with atypical paratrabecular lymphoid infiltrates, suspicious for involvement by follicular lymphoma. Negative for large cell lymphoma.

MRI and CT Neck Impression: Extensive tumor mass which likely originated within the left ethmoid sinus and extends intracranially via the cribriform plate into the anterior cranial fossa. There is involvement of the left orbit and extension into the superior aspect of the left maxillary sinuses as well as the nose. Second enhancing lesion within the right maxillary sinus measures almost 2 cm. The second mass within the floor of the right maxillary sinus, with similar imaging characteristics, is consistent with malignant involvement.

The patient has an extensive ethmoid sinus tumor, biopsy showed squamous cell carcinoma. The ethmoid sinus is not a paired organ. The patient also has a small maxillary tumor with no histologic confirmation, Hem/Oncology chart notes state the right maxillary sinus mass is carcinoma. The maxillary sinus is a paired organ.

Per the AJCC Manual (AJCC Manual for Staging, 7th edition, page 70), both the ethmoid and maxillary sinuses are further identified by their laterality (left and right). Why aren't the ethmoid sinuses a paired organ for the MP/H Rules? What MP rule applies to this case?

Malignant Central Nervous System (CNS) Rule M5 instructs us to abstract a single primary (as malignant) when a single tumor is originally diagnosed as non-malignant, the “First course treatment was active surveillance (no tumor resection),” and the subsequent resection pathology is malignant.

This patient’s first course of treatment was not active surveillance. While the patient did not have first course tumor resection, the tumor was treated with Cabergoline.

Should Rule M5 apply because there was no tumor resection? If so, should Rule M5 clearly state no tumor resection is the criteria (not active surveillance)?

SINQ 20230023 does indicate a PitNET diagnosis following a diagnosis of pituitary adenoma does not fall into standard rules, but in the previous SINQ the first course treatment was a partial resection.

It is unclear whether other types of treatment could result in a new malignant PitNET, following a previously treated non-malignant pituitary tumor.

Mantle cell lymphoma diagnosed in 1997 which was treated with chemotherapy. Now in 2014 a 'relapse' of non-Hodgkin lymphoma. They do a biopsy of the pericardium, which is called low grade B cell non Hodgkin lymphoma. See comment. The comment says histochemical stains are reviewed and findings are consistent with involvement by a CD5 positive low grade B cell lymphoma. Lack of cyclin D1 and SOX-11 positivity as well as negative IGH-CCND1 FISH analysis essentially rule out mantle cell lymphoma. The morphologic and immunophenotypic features of this disorder are not specific for any lymphoma subtype. The differential includes CLL, marginal zone lymphoma, and lymphoplasmacytic lymphoma. If this is coded NHL, NOS (9591) it is the same primary as seq. 1 and would not be abstracted.

Many interpret Rule M7 to mean when separate occurrences of TCC of the bladder are diagnosed more than 3 years apart, it is reportable as a second primary. However, doesn't Rule M6 mean that if the histology is any combination of 8050, 8120-8124 or 8130-8131 for tumors diagnosed more than 3 years apart, they are reported as a single primary?