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Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation.

Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous?

We are aware that squamous intraepithelial neoplasia, grade II (e.g., AIN II), 8077/2 is reportable for 2021. However, because this is also called rather than high grade squamous dysplasia (8077/2), we are unsure about reportability. There is no known histology and behavior code for moderate squamous dysplasia, the classifications available are only low grade (8077/0) or high grade (8077/2).

We are confused by SINQs 20180097, 20150001, and 20140051.  The latter two indicate that “well-differentiated neuroendocrine neoplasms” of the duodenum and appendix are reportable because they’re synonymous with neuroendocrine tumor (NET).  Yet 20180097 states “primary hepatic neuroendocrine neoplasm” is NOT reportable unless there is documentation that it’s being used as a synonym for Primary Hepatic Neuroendocrine Tumor (PHNET).  In addition, we see in the 2021 ICDO-3.2 update that only “poorly differentiated neuroendocrine neoplasm” is listed with behavior code /3 and noted to be reportable for 2021+ on the companion annotated histology list.  Does reportability of neuroendocrine neoplasms depend on primary site, differentiation terminology within the histology name, or something else?  Our casefinding staff is hoping for a general reportability guideline to follow when they come across “neuroendocrine neoplasms” NOS.  For example, we have a 2020 pathology report for a core biopsy of a soft tissue pelvic mass with final diagnosis of low grade neuroendocrine neoplasm; there is no further clarification as to whether it is felt to be primary or metastatic, and we have no other associated records for this patient in our central registry.

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.

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

There is a discrepancy in the SEER and STORE manual definition of code 2 for Reason for No Surgery of Primary Site. STORE includes progression of tumor prior to planned surgery as part of the definition for code 2, but the SEER Manual does not. The progression statement is included in the SEER Manual (2018 and 2021) for Reason for No Radiation, but not for Reason for No Surgery.

HL-7 e-path report, Final Diagnosis

High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment.

Comments

Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation.  Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization

Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation.

Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present.

Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type.

There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber.

Details

4/15/2018:  Right abdominal wall mass excision: infiltrating sebaceous carcinoma.  Noted to have a history of Muir-Torre/Lynch syndrome.

1/21/2019:  Two left upper back mass excisions and two lower back (laterality not specified) mass excisions: infiltrating sebaceous carcinomas

8/7/2019:  Excision of multiple sebaceous carcinomas from the right posterior back, left posterior thigh, left anterior abdominal wall, left anterior thigh, right scrotum, right lower abdominal fold, all positive for sebaceous carcinoma on pathology report

9/30/2020:  Right gluteal mass, left gluteal mass, back (NOS) excisions: sebaceous carcinomas.  

10/14/2020:  Right back excision: sebaceous carcinoma. Op note:  History of Lynch syndrome with multiple sebaceous carcinomas, recurrent back mass, site of prior mass resection.

10/18/2021:  Right thigh excision: sebaceous carcinoma

Proposed primaries using MP/H Other Sites Rules

#1:       4/15/2018:  C445-1

#2:       1/21/2019:  C445-2, separate from #1 per M8, same as 1/21/19 C445-9 per M18

#3:       8/7/2019:  C445-1, separate from #1 per M10, separate from #2 per M8

#4:       8/7/2019:  C447-2, separate from #1 & #3 per M8, separate from #2 per M12

#5:       8/7/2019:  C632, separate from #1 per M10, separate from #2-#4 per M11

#6:       9/30/2020:  C445-2, separate from #1 & #3 per M8, separate from #2, #4 & #5 per M10

#7:       9/30/2020:  C445-1, separate from #2, #4 & #6 per M8, separate from #1, #3 & #5 per M10; I do not think the back, NOS (C445-9) is a new primary per M18.

#8:       10/18/2021:  C447-1, separate from #2, #4 & #6 per M8, separate from #1, #3, #5 & #7 per M10