UNASSIGNED: A 55-year-old male patient developed a mass in the left inguinal area with left lower limb swelling and first visited a local hospital 3 months earlier because of unrelieved pain. An MRI scan suggested left suprapubic branch and left acetabular bone destruction, abnormal soft tissue signals within the iliopsoas muscle of the anterior edge of the left iliac bone, and enlarged lymph nodes in the left iliac fossa and left inguinal region. The patient subsequently underwent left pelvic lesion open biopsy and inguinal lymph node resection biopsy. According to pathological reports, the left inguinal mass was considered to be a malignant tumor of cutaneous accessory origin (pilomatrix carcinoma) with extensive vitreous changes. The suprapupubis branch mass was considered to be a bone metastatic pilomatrix carcinoma. Immunohistochemistry (IHC) revealed a PDL1 combined positive score (CPS) of 8. DNA next-generation sequencing (NGS) showed CDKN2A L65Rfs*53 mutation. The patient received three cycles of gemcitabine and nedaplatin. However, the lesion progressed.
UNASSIGNED: Chemotherapy is not effective for treating pilomatrix carcinoma. PDL1 antibodies and CDK4/6 inhibitors might be treatment options for pilomatrix carcinoma.

暂无摘要。

BACKGROUND: Pilomatrix carcinoma (PC) is a rare skin malignancy of the hair follicles matrix that tends to be locally aggressive with 10-16 % risk of metastasis mostly to the pulmonary and lymphatic system. There are no clear protocols for the management of PCs, however surgical intervention with clear margins has been highly considered in PC management to decrease risk of recurrence.
METHODS: A 40 year-old male patient presented to our clinic to evaluate an asymptomatic, slow-growing nodule localized on his left thigh. A CT scan revealed a well-defined, enhanced lesion with microcalcification. \"En bloc\" surgical resection of the lesion was performed and histopathology confirmed the diagnosis of pilomatrix carcinoma.
UNASSIGNED: Given its rarity, there are no definitive guidelines regarding PC treatment. However, surgical intervention with clear margins including wide local excision or Mohs micrographic surgery has been highly considered. In our case, wide excision of the lesion with clear margins was performed with no evidence of recurrence one year later.
CONCLUSIONS: Given the local aggressive nature of PC, appropriate surgical intervention is essential in decreasing the risk of recurrence. Wide excision with clear margins has been proposed to decrease the risk of recurrence. Additionally, total-body skin examination should be done 2-3 times annually to evaluate for recurrence or metastasis.

UNASSIGNED: A very rare skin cancer. Malignant variant of pilomatricoma. It is unique because of its highly aggressive behavior. Responds poorly to chemotherapy and radiotherapy. Treated with a wide surgical excision with safe margins.
UNASSIGNED: Pilomatrix carcinoma is uncommon and locally aggressive tumor of the hair follicle matrix. It is a malignant variant of pilomatricoma. If left untreated for a long time may attain massive size and has the potential for distant spread. Only few cases with brain extension have been reported in the literature. Diagnosis of these tumors is established by histopathology. Although pilomatrixoma and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. The tumors respond poorly to chemotherapy and radiotherapy. Thus, recommended treatment is a wide surgical excision with safe margins. Herein, we report the case of a 39-year-old African woman with pilomatrix carcinoma of the scalp eroding the skull bone with intracranial extension.

Pilomatrix carcinoma is a rare tumor arising from the hair follicle matrix cells most commonly seen in the head and neck region. Also known as \"calcified epithelial carcinoma of Melherbe,\" it was first reported in 1980 by Lopansri and Mihm. Since then till date to the best of our knowledge only around 125 cases were reported in literature, of which only 11 cases were reported to arise from histologically proven areas of previous pilomatrixoma which is the benign variant. One such case is being reported here along with the review of literature. A 50-year-old man presented with a swelling in the nape of his neck since 6 months, which was gradually increasing in size. He had a history of similar swelling at the same site 18 months back for which he underwent a surgery at a center outside. Final histopathology report was suggestive of pilomatrixoma with negative margin. Wide local excision of the tumor with 3 cm margin, placement of surgical clips followed by a primary closure was done. The final histopathology report is suggestive of pilomatrix carcinoma. The patient has no recurrence in 6 months follow-up. The differential diagnosis of pilomatrix carcinoma should be considered in cases of recurrent skin tumors. Wide local excision is the preferred treatment. Re-excision should be done in margin positive cases and cases where simple excision was done due to improper preoperative diagnosis. Due to the rarity of the disease, adjuvant treatment is not properly defined.

BACKGROUND: Pilomatrix carcinoma is a rare malignant neoplasm arising from the root of hair follicles, with only 150 cases described in the world literature. It is most commonly seen in the head and neck region.
METHODS: We describe a case of malignant pilomatrix carcinoma in a 62-year-old gentleman presenting as a solitary globular mass over the right anterior chest wall along with a brief review of literature.
CONCLUSIONS: Surgical excision with a wide margin is the current standard of care for chest wall pilomatrix carcinoma and is associated with the least recurrence. Role of radiation as definitive treatment of the primary or as adjuvant therapy has not been clearly established.

Pilomatrix-like high-grade endometrioid carcinoma (PiMHEC) has recently been described as an aggressive variant of endometrial carcinoma. Herein, we described a case of ovarian PiMHEC, comparing it to endometrial PiMHEC and assessing previously published cases of putative ovarian PiMHEC. A 65-year-old woman underwent hysterectomy for an ovarian tumor characterized by solid nests of basaloid cells with prominent ghost cell keratinization. Immunohistochemistry showed nuclear β-catenin and CDX2 expression and loss of estrogen and progesterone receptors and PAX8. These features were consistently observed in all previously published cases and may represent diagnostic criteria of PiMHEC. Other frequent features were geographic necrosis and a low-grade endometrioid component. CK7, neuroendocrine, and basal/squamous markers were inconsistently expressed. All cases with available follow-up showed poor prognosis. PiMHEC should be distinguished from mimickers, such as high-grade endometrioid carcinoma with geographic necrosis, low-grade endometrioid carcinoma with ghost cell keratinization, and undifferentiated/dedifferentiated carcinoma. In conclusion, PiMHEC can also occur in the ovary and shows several consistent clinical, morphological, and immunophenotypical features. These features support that PiMHEC is a distinct entity requiring an aggressive management.

Malignant pilomatricoma is a rare cutaneous malignancy that is commonly found on the head and neck. We present a patient with malignant pilomatricoma of the lower extremity with intralesional calcification and giant cells, confirmed by histopathology. This patient\'s case represents a clinically important variation of malignant pilomatricoma in an uncommon anatomical location.

An 80-year-old female presented with a slowly growing 2-cm nodule on her shoulder over a 1-year period. Histopathologic sections of a biopsy specimen showed a multinodular, dermal-based basaloid tumor with areas of clear-cell change, stromal induction, as well as significant cytologic atypia and atypical mitotic activity. An initial investigation revealed positive staining of CDX2, a well-known marker of tumors of gastrointestinal origin. The case was referred to our dermatopathology service for consultation to determine if the lesion was in keeping with a cutaneous metastasis. On receipt of the case, an extended immunohistochemical panel was performed including SATB2, which displayed a similar pattern of staining as seen with CDX2. Although pathologists are most familiar with CDX2 and SATB2 as markers of gastrointestinal origin, the recent dermatopathology literature highlights that primary adnexal lesions of the skin also display positivity for CDX2 and can exhibit SATB2 positivity. We share a case of pilomatrix carcinoma with positive expression of nuclear CDX2 and SATB2, adding to the recent literature to (a) increase recognition of this staining pattern in hair follicle tumors, and (b) discuss briefly the shared molecular underpinnings in the tumorigenesis of gastrointestinal tumors and tumors of hair follicle origin that help clarify this underrecognized immunohistochemical pattern.

Pilomatrixoma is an uncommon, benign tumor with differentiation towards both the hair matrix and cells arising in the cortex, most frequently appearing in the first or second decade of life. In rare instances, pilomatrixomas can show malignant transformation. Pilomatrix carcinoma is extremely uncommon and has traditionally been considered a tumor of low malignant potential; however, a high local recurrence rate has been reported. There is a paucity of literature on these lesions, with only a few reports describing the spectrum of malignant changes seen in these lesions. In this case report, we present a case of pilomatrixoma in an adult patient showing atypical features. While the tumor is small, there are focal features that suggest progression to malignancy, but do not fulfill the criteria for pilomatrix carcinoma. These focal atypical features include a focal infiltrative pattern at the periphery, with a variable cytological atypia and an increased mitotic rate, up to five mitotic events/high-power field. Irregular foci of central necrosis (comedonecrosis) were present in several lobules. Some of the features identified were similar to a subset of pilomatrixoma, known as \"proliferating pilomatrixoma.\" However, our case did not have the diffuse changes or larger size that has been frequently reported in \"proliferating pilomatrixoma.\" In conclusion, given the lack of focality of the changes, the lesion in our case is best described as a pilomatricoma with atypical features. Furthermore, our case may highlight the need to ensure close clinical follow-up for these lesions with unexpected atypical features that raise concern of recurrence and malignant transformation.