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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?

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?

Pathology report states: the individual nodules of trichoblastic cells resemble those seen in trichoblastoma, but the lesion is very poorly circumscribed with an infiltrative border that extends into the subcutis. the lesion may behave in a locally aggressive fashion, and should be completely removed. High grade trichoblastic carcinomas can metastasize.

According to the literature, PMH is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. However, the references also state: PMH is a cutaneous tumor that behaves in an indolent fashion. There is no indication that this was a malignant diagnosis.

12/3/18 Foot, left skin lesion, punch biopsy: Superficial squamous epithelium demonstrating hyperkeratosis and fragments of keratin debris, no tumor seen.

Foot, left skin lesion, punch biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

NOTE: The submitted immunohistochemical slides were reviewed. Positive and negative controls reacted appropriately. The tumor cells demonstrate immunoreactivity to CK AE1/AE3 and CK7. The CD31 immunoreactivity described in the report cannot be confirmed as only the positive control is submitted for review. The tumor cells are negative for desmin, CD45, CD68, S-100, CD34, SMA, CD20, and HHV8. The proliferative index via Ki-67 is approximately 10%. The morphology (described below) and immunohistochemistry performed are compatible with a pseudomyogenic hemangioendothelioma.

12/4/18 Final Pathologic Diagnosis: Foot, left bone lesion, biopsy: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma, see note.

Note: The patient's imaging findings were reviewed in conjunction with this case, revealing numerous lytic lesions of the tibia, fibula, talus, tarsal, metatarsal, and phalangeal bones. Additionally, as per the medical record, also reviewed in conjunction with this case, there are lesions of the skin. Thus, an extensive immunohistochemical panel was performed in an attempt to support the morphologic findings in this case, which were morphologically similar to the patient's skin biopsy. The tumor cells demonstrate strong immunoreactivity to pancytokeratin (CK AE1/AE3) and vimentin with moderate immunoreactivity to Fli-1. The tumor cells demonstrate weak immunoreactivity to epithelial membrane antigen. INI-1 is retained. There is focal immunoreactivity to CD31 although this is limited to the edges of the tissue fragments. The tumor cells are negative for HHV-8, CD34, smooth muscle actin, CK8/18, desmin, CD99, and Bcl-2. The combination of morphologic (see below for microscopic description) and immunohistochemical findings are consistent with pseudomyogenic hemangioendothelioma. Fresh tissue was submitted for karyotype analysis at the time of intraoperative consultation; however, it revealed only a normal appearing male karyotype. Thus, molecular confirmation was sought. The original slides and a paraffin block were submitted for FOSB rearrangement analysis, as pseudomyogenic hemangioendothelioma is known to have recurrent rearrangements with FOSB. Additional immunohistochemistry performed at (FACILITY) demonstrating immunoreactivity for ERG, supporting a vascular origin for this neoplasm. Fluorescence in situ hybridization demonstrated that 13% of the cells examined show FOSB rearrangement. While this FISH probe is for investigational purposes, the above findings support the diagnosis of pseudomyogenic hemangioendothelioma.

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.