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2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine.

Patient is 3 years old.

07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney.

10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis.

The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy.

It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable.

While we recognize that paragangliomas originate in the parasympathetic or sympathetic nervous system, these are endocrine tumors and endocrine glands/structures are not included in ICD-O site code C479 (Autonomic nervous system). Endocrine tumors of the paraganglia have their own site codes (C75_) per the ICD-O. Additionally, the ICD-O-3.2 provides specific sites for most of the paragangliomas included in Table 9. Per the ICD-O-3.2, carotid body paraganglioma is C754, and middle ear paraganglioma, glomus jugulare tumor, jugulotympanic paraganglioma, and paraganglioma (NOS) are C755.

Why are paragangliomas not coded to the paraganglia sites (C75_) provided in the ICD-O? Should these sites be added to the Head & Neck schema for the specific paragangliomas arising in the head and neck? Obtaining consistency in coding primary site for these tumors will be difficult if registrars use the ICD-O provided site codes instead of the primary site statement preceding Table 9. Additionally, as most registrars may use the ICD-O provided site code, the Head and Neck schema in the Solid Tumor Rules would not apply, the Other Sites schema would apply to sites C754 and C755.

Scenario: Patient had a transurethral resection of the prostate on 8-29-19, positive for basal cell carcinoma with focal squamous differentiation involving approximately 50% of tissue (determined not to be mets by consult). On 11-14-19, the patient had a prostatectomy positive for residual basal cell carcinoma and a small focus of infiltrating prostatic adenocarcinoma. According to AJCC, 8th edition, page 724, basal cell carcinoma of the prostate is 8147/3 and we ignored the small focus of adenocarcinoma. The above scenario was reported as two primaries (8090/3 and 8140/3), but we are thinking it is one.

Patient was diagnosed with low-grade non-Hodgkin lymphoma in 2011, later classified as hairy cell leukemia-variant.

Right cervical node biopsy in 2020 proved HCL-v and a subsequent 2021 right tongue base biopsy showed DLBCL. The tongue base biopsy path includes the comment, patient has history of HCL-v, but the morphology and flow cytology features are different from the patient's previous right cervical node biopsy. This DLBCL likely represents a second de novo lymphoma, but cannot exclude an unusual transformation of the prior HCL-v.

Per Heme Rule M7, abstract a single primary when a more specific histology is diagnosed after an NOS if the Heme DB confirms the same primary. The histology code for HCL-v, 9591/3 is a non-specific code, but it seems like a specific histology. The Heme Calculator does say 9591 and 9680 are the same primary, but we are unsure if that is correct for this case of HCL-v followed by DLBCL.

Rule H8 of the Melanoma Solid Tumor Rules states that multiple variants of melanoma in one tumor are rare and a question must be submitted to Ask a SEER Registrar (AASR) for the correct histology code. However, our facility has seen a number of these cases in 2021 and would like to track the official answer and make it available to all in this format.

How should histology be coded for the following?

1. January 2021 diagnosis of left shoulder invasive malignant melanoma, histologic type: nodular and desmoplastic types per College of American Pathologists (CAP) summary of punch biopsy.

2. May 2021 shave biopsy of left arm invasive malignant melanoma, superficial spreading and nodular variant is listed in the CAP summary.

3. June 2021 diagnosis of right cheek invasive malignant melanoma, histologic subtype: superficial spreading and nodular seen on CAP summary of shave biopsy.

Autopsy/Pathology Report (2020) excerpts

External Examination

Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area.

Final Anatomic Diagnosis

Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies)

Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated

Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified.

MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS.

The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary.

Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC).

Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction.

If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11.