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In the 2018 ICD-O-3 Update Table, undifferentiated high-grade pleomorphic sarcoma and undifferentiated high-grade pleomorphic sarcoma of bone (C40_) were both listed as a New Term for histology 8830/3. There was no site restriction for a diagnosis of undifferentiated high-grade pleomorphic sarcoma. Therefore, it appears the diagnosis could easily be applied to a soft tissue tumor. This histology is used by pathologists in our region for soft tissue tumors as well as bone tumors. However, in the ICD-O-3.2 Table an entry (or synonym) was not provided for a tumor outside the bone.

The ICD-O-3.2 Table only lists undifferentiated high-grade pleomorphic sarcoma of bone for site codes C40_ and C41_ as a synonym for histology 8830/3. This also is not listed in the ICD-O-3.2 Implementation Guidelines. As a result, it is unclear whether a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue can be coded to 8830/3 and/or can be a synonym for the preferred term (8830/3, Malignant fibrous histiocytoma). Can a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue be coded to 8830/3, C49_ as it was per the 2018 ICD-O-3 Update Table?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

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

Example: Patient has left thigh skin excision with final diagnosis of atypical smooth muscle cell proliferation, inked peripheral margin is involved and inked deep margin is free of disease in the sections examined. See Comment.

Diagnosis comment states: The terminology regarding this lesion is controversial. Lesions with identical features are designated as leiomyosarcoma in the dermatopathology literature, whereas, the preferred classification in the soft tissue pathology is atypical intradermal smooth muscle neoplasm. Although the lesion appears predominantly dermal based, since the margin is involved, the lesion cannot be entirely evaluated, and therefore the final designation is deferred to the findings in the excisional specimen. (This slide was read by bone and soft tissue pathologist.)

There has been no excision of this tumor and, as a central registry, we have no access to the pathologist for clarification.

Is this skin case reportable based on the dermatopathology interpretation when further documentation is not available?

Example: Operative report states, partial simple vulvectomy, anoscopy with normal-appearing clitoris, clitoral prepuce, bilateral labia majora, and labia minora. There is a 1.5 x 1 cm raised, hyperpigmented lesion which appears consistent with VIN 3 on the perineal body, just to the right of midline, and not touching the midline. It goes quite close to the anus but is not touching the anus.

Final diagnosis on resection is, Invasive squamous cell carcinoma arising in a background of high-grade squamous intraepithelial neoplasia (VIN III) with the following features: Location: perineum. Focal invasion arising in setting of 1 cm area of VIN III.

Patient had a skin biopsy and this is the interpretation: NASAL SUPRATIP: INVASIVE BASAL CELL CARCINOMA OF SKIN WITH NEUROENDOCRINE DIFFERENTIATION

NOTE: The deep margin is positive for tumor; peripheral margins negative for tumor. The tumor has a basaloid appearance with focal areas appearing slightly squamoid, and it demonstrates myxoid/mucinous retraction from the stroma. It does not demonstrate peripheral palisading of cells within tumor nests and has nuclear chromatin which suggests neuroendocrine differentiation. Mitotic rate is more brisk than typical basal cell carcinoma as well. The differential diagnosis includes basal cell carcinoma with or without neuroendocrine differentiation, basal cell carcinoma with squamous differentiation, basaloid squamous cell carcinoma, Merkel cell carcinoma and metastatic small cell carcinoma. The tumor is further characterized per immunostains x 9 (controls work well). Tumor cells are positive for Ber EP4 and p63; focally positive for Chromagranin; while negative for EMA, CK20, CK7, TTF-1, CD56 and Synaptophysin. Overall, the staining pattern supports basal cell carcinoma with neuroendocrine differentiation.