Primary Cutaneous Carcinosarcoma Research Articles

A primary cutaneous carcinosarcoma of the retro auricular region, how to treat and literature review.

Carcinosarcomas are malignant biphasic tumours of epithelial and mesenchymal tissue. They are most often found in visceral organs, but also appear on the skin. Older age, male sex and chronic sun exposure are risk factors for its development. In this article we report a case and provide a review of literature regarding primary cutaneous carcinosarcoma (CCS) with special regard to its management. A manual electronic search of the PubMed Medline and Web of Science Core Collection databases was performed encompassing all included reports until 30th November 2022 to identify studies that reported primary CCS. CCS is a rare and aggressive tumour. Diagnosis requires histological examination and immunoreactivity of epithelial and mesenchymal components to specific markers. On its diagnosis, possibility of metastasis of a visceral carcinosarcoma should always be excluded. Surgical excision with clear margins, including the use of Mohs micrographic surgery (MMS), is the primary treatment for CCS. Reconstruction the excision defect should be performed. Regular follow-up for 5 to 10 years after initial treatment is advised. Awareness for CCS is necessary in the diagnostic evaluation of skin tumours. Further research is needed to better understand the underlying mechanisms of CCS and to establish optimal management strategies for this challenging malignancy. We recommend complete surgical excision using MMS as the treatment modality for this type of skin cancer. Dermatological follow-up for at least 5 years should be conducted to monitor for recurrence.

Primary Cutaneous Carcinosarcoma Treated With Mohs Micrographic Surgery in a Hospice Patient.

Primary Cutaneous Carcinosarcoma Treated With Mohs Micrographic Surgery in a Hospice Patient.

Primary cutaneous carcinosarcoma: report of a case with poroid and fibrohistiocytic differentiation.

Primary cutaneous carcinosarcoma is an infrequent cutaneous biphasic tumor composed of an epithelial and a mesenchymal component, both with malignant features. The epithelial component frequently shows squamoid or basaloid differentiation but other differentiations, such as adnexal, have also been described. The mesenchymal component shows diverse cellular lineages such as smooth muscle, nervous, osteoblastic, chondroblastic, myofibroblastic, angiomatous, and fibrosarcomatous among others. Local recurrences with low metastatic potential are also reported. We present the case of an 89-year-old man with an exophytic tumor located in the back of the left leg. The biopsy showed a cutaneous carcinosarcoma with adnexal differentiation (porocarcinoma) and pleomorphic sarcoma with fibrohistiocytic differentiation.

Temporal primary cutaneous carcinosarcoma in the ear: A case report

Carcinosarcoma is a rare biphasic tumor made of two malignant components—the epithelial component and the stromal component—that can develop anywhere on the body, but mainly in sun-exposed areas. We report the case of a 78-year-old male who presented himself with a right temporal tumor in the ear 10 cm in diameter. A biopsy suggested a sarcoma. The patient underwent an extensive surgical resection of the temporal mass and the ipsilateral ear. A microscopic examination of the tumor revealed two intermixed malignant contingents. The epithelial component was made of atypical basaloid cells arranged in nests with peripheral palisading and expressing cytokeratin. The stromal component was made of atypical spindle cells expressing smooth muscle actin (SMA). A diagnosis of primary cutaneous carcinosarcoma with clear margins was reached. The patient is alive and without recurrence after twelve months of a follow-up period.

Primary cutaneous carcinosarcoma: clinical, histological, and immunohistochemical analysis of eight cases.

Cutaneous carcinosarcoma is a rare biphasic tumor comprising malignant epithelial and heterologous mesenchymal elements. Data on the clinical and histopathologic characteristics of this tumor are scarce. The objective of this study was to describe the clinicopathologic and immunohistochemical features of cutaneous carcinosarcoma. A descriptive retrospective study was conducted in a tertiary care hospital from Spain. We reviewed the records of eight patients with cutaneous carcinosarcoma who were diagnosed from 2009 to 2019. The mean patient age at diagnosis was 72.13years (range 44-91years), and there was a male predilection (6 cases). The most common site of cutaneous carcinosarcoma was the head and neck (5 cases). Carcinosarcomas demonstrated variable histopathological and immunohistochemical features. Follow-up was available for 7-8 patients. There were two cases of local recurrence and one case of metastasis. Two patients died from the tumor during the entire follow-up. Although the number of cases in this study was limited, our results provide valuable insight into the clinical, histopathologic, and immunohistochemical characteristics of primary cutaneous carcinosarcoma.

Primary Carcinosarcoma of the Skin

Primary cutaneous carcinosarcoma is a biphasic tumour containing both malignant epithelial and malignant mesenchymal elements. The disease is a rare malignant skin tumour of unknown aetiology with significant potential to recur locally and to metastasise. Surgery is the primary therapeutic modality. We describe an unusual case of primary cutaneous Carcinosarcoma localised in the hand.

Primary cutaneous carcinosarcoma: insights into its clonal origin and mutational pattern expression analysis through next-generation sequencing

Primary cutaneous carcinosarcoma is a rare biphenotypic neoplasm exhibiting both epithelial and sarcomatous elements. Even though its origin and biological aspects remain poorly understood, it has been postulated that this tumor may arise from progenitor cells, which subsequently differentiate into distinct tumor components. We have investigated the histological and immunohistochemical staining patterns of a cutaneous carcinosarcoma case, as well as its ultrastructural aspects. In addition, sarcomatous and epithelial tumor components were separated by laser capture microdissection and subjected to targeted, high-depth, next-generation sequencing of a 46-cancer gene panel to asses the gene mutational pattern amongst both components. There were transitional cells at the epithelial/mesenchymal transition that labeled with putative progenitor cell markers (K19, c-kit, CD34 and Bcl-2). There was shared reactivity to antibodies directed against the progenitor cell marker EpCAM (epithelial cell adhesion molecule) in both components. Ultrastructurally, individual cells were demonstrated to have overlapping features of epithelial and mesenchymal differentiation. The mutational analysis revealed point mutations in exon 5 of TP53, which were identical in both the epithelial and sarcomatous components, and which were concordant with p53 expression at a tissue level. The aforementioned histological, ultrastructural, immunohistochemical and mutational pattern is strongly suggestive of a common clonal origin to the distinct elements of this tumor.

Primary Cutaneous Carcinosarcoma of the Shoulder: Case Report with Literature Review

Primary Cutaneous Carcinosarcoma of the Shoulder: Case Report with Literature Review

Primary cutaneous carcinosarcoma: Dermoscopic and immunohistochemical features

A 73-year-old man presented a 9-month history of an enlarging nodule on his right temple. Dermoscopy revealed a non-pigmented lesion with ulceration, fibrosis and pale globules. An excisional biopsy was carried out and histology showed a biphasic tumour with a basal cell carcinoma like epithelial component and a dermal undifferentiated sarcoma, with pleomorphic spindle cells and numerous osteoclast-like giant cells. Based on immunohistochemistry findings, a diagnosis of primary cutaneous carcinosarcoma was made and the patient underwent wide local excision.

Cutaneous Carcinosarcoma With Myoepithelial Differentiation: Immunohistochemical and Cytogenetic Analysis of a Case Presenting in an Unusual Location

Primary cutaneous carcinosarcoma is an uncommon tumor that is characterized by an admixture of malignant epithelial and mesenchymal elements. To our knowledge, only approximately forty cases have been reported to date in the literature. The majority of these neoplasms occurred on the extremities and the head and neck region of older individuals. We report an additional case, which presented in an 87-year-old man in an unusual location, the buttock. Histologically, the lesion was characterized by a biphasic proliferation of malignant epithelial areas admixed with an infiltrative, poorly differentiated spindle cell component. The epithelial areas consisted of nodular aggregates of basaloid to squamoid cells forming focal ductal structures with central lumens containing proteinaceous debris. The sarcomatoid component consisted of malignant spindle to epithelioid-appearing cells admixed with bizarre-appearing tumor giant cells within a myxochondroid stroma. The epithelial cells showed positive staining with pan-cytokeratin, BerEP4, p63, and keratin 5/6 and were focally positive for S-100, calponin, and actin, consistent with myoepithelial differentiation. An epithelial membrane antigen stain highlighted the scattered ductal structures. The high-grade spindle cell areas were negative for S-100, actin, desmin, calponin, p63, epithelial membrane antigen, and keratin 5/6.

P63 is a helpful tool in the diagnosis of a primary cutaneous carcinosarcoma

Journal of Cutaneous PathologyVolume 36, Issue 2 p. 280-282 P63 is a helpful tool in the diagnosis of a primary cutaneous carcinosarcoma Paolo Romanelli, Paolo Romanelli Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorMaria Miteva, Maria Miteva Department of Dermatology and Venereology, Medical University Sofia, Sofia, BulgariaSearch for more papers by this authorElissa Schwartzfarb, Elissa Schwartzfarb Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorCarlos Ricotti, Carlos Ricotti Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorTory Sullivan, Tory Sullivan Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorPascual Abenoza, Pascual Abenoza Dermpath Diagnostics, South Florida, FL, USASearch for more papers by this authorMehrdad Nadji, Mehrdad Nadji Department of Pathology, University of Miami School of Medicine, Miami, FL, USAe-mail: [email protected]Search for more papers by this author Paolo Romanelli, Paolo Romanelli Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorMaria Miteva, Maria Miteva Department of Dermatology and Venereology, Medical University Sofia, Sofia, BulgariaSearch for more papers by this authorElissa Schwartzfarb, Elissa Schwartzfarb Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorCarlos Ricotti, Carlos Ricotti Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorTory Sullivan, Tory Sullivan Department of Dermatology and Cutaneous Surgery, University of Miami School of Medicine, Miami, FL, USASearch for more papers by this authorPascual Abenoza, Pascual Abenoza Dermpath Diagnostics, South Florida, FL, USASearch for more papers by this authorMehrdad Nadji, Mehrdad Nadji Department of Pathology, University of Miami School of Medicine, Miami, FL, USAe-mail: [email protected]Search for more papers by this author First published: 20 January 2009 https://doi.org/10.1111/j.1600-0560.2008.01046.xCitations: 10Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume36, Issue2February 2009Pages 280-282 RelatedInformation

Primary cutaneous carcinosarcoma: case report with expanded immunohistochemical analysis

An 83‐year‐old woman presented with a nodular, eroded tumor on the skin between the nose and the upper lip of 18 months’ duration. There were no palpable lymph nodes and no infiltrates on chest radiography. Complete surgical excision showed a tumor measuring 65 × 40 × 30 mm. On histopathologic examination, it was composed of typical basal cell carcinoma (BCC) nodules and large sheets of oval or short spindle cells (Fig. 1a), with vesicular nuclei, distinct nucleoli, moderate pleomorphism, and pronounced mitotic activity (more than 40 mitoses/10 high‐power fields). In parts abutting the upper lip, BCC nodules were found in the muscle layer, but the small salivary glands were uninvolved (Fig. 1b). Immunohistochemical analysis (Table 1) revealed a cytokeratin (CK)‐positive, Ber‐EP4‐positive, and vimentin‐negative BCC component (Fig. 2a), and a vimentin‐positive, CK‐negative sarcomatous component (Fig. 2b). In addition, mesenchymal tumor components were focally positive for smooth muscle actin (SMA). The BCC component showed irregular reaction with CK7, which stained some lobules and parts of individual nests (Fig. 2c). The final diagnosis was primary cutaneous carcinosarcoma. At the last visit, 3 months after operation, no signs of recurrence or metastatic spread were observed. Additional immunohistochemical analyses showed preserved membranous β‐catenin staining in the BCC component, without nuclear reaction in the mesenchymal component. The labeling index (LI) is expressed as the percentage of positive cells, and is calculated from the number of positive tumor cells divided by the total number of tumor cells counted (minimum 300 cells) in the areas with most pronounced immunopositivity. Counting was performed on images taken from microscopic high‐power fields with an Olympus DP70 digital camera (Olympus Corporation, Tokyo, Japan). The program analySYS (Soft Imaging System, Munster, Germany) was used, with the screen grid and the manual touch‐count method. The LI values of Ki‐67 and telomerase reverse transcriptase (hTERT) were higher in the sarcomatous component. hTERT displayed enhanced nucleolar localization and diffuse staining of mitotic cells. Histone deacetylase 1 (HDAC1) was expressed in a smaller percentage of cells than HDAC2, with a higher LI in the sarcomatous component. HDAC2 was the only marker analyzed that stained more cells in the BCC component (Fig. 2d).(a) Basal cell carcinoma (BCC) nodules and diffuse proliferation of sarcomatous cells. (b) Deep peripheral parts of the tumor show BCC nodules infiltrating into the muscle layer, but the small salivary glands are uninvolved. Hematoxylin and eosin; original magnification: (a) ×100; (b) ×40image Immunohistochemical staining in carcinosarcoma Antigen Source Epithelial (BCC) component Mesenchymal component CK (AE1/AE3) Dako* + – EA (Ber‐EP4) Dako + – CK7 Dako + (variable) – EMA Dako – – CEA Dako – – S‐100 Dako – – HMB‐45 Dako – – Vimentin Dako – + Factor VIII‐related Ag Dako – – CD34 Dako – – CD68 Dako – – CD99 Dako – – LCA Dako – – α‐SMA Dako – + (focal) Desmin Dako – – β‐Catenin Santa Cruz† + (membranous) – Ki‐67 (LI) Dako 10% 32% hTERT (LI) Novocastra‡ 22% 41% HDAC1 (LI) Santa Cruz 16% 40% HDAC2 (LI) Santa Cruz 76% 37% CEA, carcinoembryonic antigen; CK, cytokeratin; EA, epithelial antigen; EMA, epithelial membrane antigen; HDAC, histone deacetylase; hTERT, telomerase reverse transcriptase; LCA, leukocyte common antigen; LI, labeling index; SMA, smooth muscle actin. Glostrup, Denmark. Santa Cruz, CA, USA. Newcastle, UK. (a) Cytokeratin (CK) stain reveals epithelial (basal cell carcinoma, BCC) nodules, whereas the large sheet of sarcomatous cells (top left) is unstained. (b) Vimentin‐positive sarcomatous component and negative BCC nodules. (c) Variations of CK7 staining between BCC nodules and inside individual nodules. (d) Histone deacetylase 2 (HDAC2) expression is higher in the BCC component (right) than in the sarcomatous component. Streptavidin–biotin; original magnification: (a) ×12.5; (b, c) ×100; (d) ×400image

P63 in Primary Cutaneous Carcinosarcoma

Primary cutaneous carcinosarcomas (PCCs) are rare malignant neoplasms that are characterized by biphasic epithelial and mesenchymal differentiation. When the biphasic nature is not evident, immunohistochemical studies may be important in the diagnosis of PCCs. Although AE1/AE3 is frequently used to demonstrate the epithelial component, it may not be strongly expressed in epithelial cells that are not well-differentiated. p63 is a protein homologue of p53 that is expressed in poorly differentiated epithelial cells. We report 3 cases of PCC. The clearly epithelial areas of each tumor were frequently positive for both markers, whereas the sarcomatous areas were negative for both markers. Epithelial cells that were poorly differentiated and not easily identifiable were positive for p63 but negative for AE1/AE3. Of interest, transitional areas showed positivity for p63 alone. These 3 cases suggest that the use of both p63 and routine cytokeratin markers such as AE1/AE3 can increase the sensitivity for distinguishing epithelial cells over a range of differentiation states, which we propose will aid in the diagnosis of PCCs. In addition, the staining pattern of AE1/AE3 and p63 in these cases further supports the conversion theory of PCC.

Recurrent cutaneous tumour with features of an osteosarcoma: Primary osteosarcoma or cutaneous carcinosarcoma?

We report the case of a recurrent tumour with histological features of an osteosarcoma, and discuss the differential diagnosis.An 80 year old woman presented with a fungating tumour on the right calf, arising from an area of previous split skin grafting. There was also a new 1–2 cm diameter nodule at the medial ankle of the right leg. The patient had previously had a lesion removed from the same site, reported as a squamous cell carcinoma. The recurrent calf lesion was excised, as well as the skin nodule on the medial ankle of the right leg. Histological examination of both specimens showed similar features, with a malignant neoplasm having features of an osteosarcoma, with osteoid formation. No carcinomatous component was seen in the sections examined. Immunohistochemical studies for cytokeratin, S100, HMB45 and Melan‐A were negative in the malignant cells. Tumour giant cells were CD68 positive.The differential diagnoses included recurrent squamous cell carcinoma presenting as a carcinosarcoma with sarcomatous overgrowth, primary cutaneous osteosarcoma, metastatic osteosarcoma, malignant mixed dermal tumour, malignant peripheral nerve sheath tumour with an osteosarcomatous component and undifferentiated pleomorphic sarcoma with osteosarcoma‐like areas.Primary cutaneous carcinosarcomas, primary cutaneous osteosarcomas and metastatic osteosarcoma to the skin are all rare. Squamous cell carcinomas and basal cell carcinomas are the most common epithelial component of cutaneous carcinosarcomas, with osteosarcomas and undifferentiated pleomorphic sarcomas (malignant fibrous histiocytomas) the most commonly reported sarcomatous component. Extraskeletal osteosarcomas are also uncommon, with the skin a rarely reported primary site. Predisposing factors for extraskeletal osteosarcomas include past trauma, pre‐existing soft tissue calcification, ossification, myositis ossificans and intramuscular injection sites.This is an unusual presentation of what would appear to be a recurrent tumour, previously diagnosed as squamous cell carcinoma. The histological appearance raises a number of possibilities, requiring clinicopathological correlation.

Are primary cutaneous carcinisarcomas underdiagnosed? Five cases and a review of the literature

Primary cutaneous carcinosarcoma is a biphasic tumour containing both malignant epithelial and malignant mesenchymal elements. To date, only 26 cases have been reported in the literature. However, our findings suggest that this may reflect underreporting and possibly underdiagnosis. We present five cases and a comprehensive review of the literature: The disease most commonly presents in the eighth and ninth decades of life, is twice as common in males and may be related to sun exposure. Surgery is the primary therapeutic modality. Despite treatment, 27% of cases developed metastatic disease. Both epithelial and mesenchymal elements have been implicated in disease spread. We hope to raise awareness of this uncommon but serious disease and to assist clinicians in its management.

Primary cutaneous carcinosarcoma arising in a patient with nevoid basal cell carcinoma syndrome

Nevoid basal cell carcinoma syndrome (NBCC) is an autosomal dominant disorder characterized by developmental abnormalities and neoplasms including basal cell carcinoma (BCC) and sarcomas (i.e. leiomyosarcoma, rhabdomyosarcoma, and fibrosarcoma). Primary cutaneous carcinosarcoma (PCC), a rare tumor composed of malignant epithelial and mesenchymal components, has never been previously described in association with this syndrome. A 61-year-old Hispanic man with a history of NBCC presented with a 4 cm nodule on the right proximal medial thigh. PATHOLOGIC FINDINGS: Areas of typical BCC merged with intersecting fascicles of large atypical spindle cells that stained for vimentin and were negative for actin, desmin, CD-34, and S-100 protein. Scattered bizarre solitary cytokeratin-positive epithelioid cells were embedded within the fibrocytic proliferation. Several carcinosarcomas have been reported to contain BCC as the malignant epithelial component, but to our knowledge, this is the first report of PCC associated with NBCC. Mutation in patched tumor suppressor gene on chromosome 9q occurs in BCCs of NBCC, and aberrancies on chromosome 9q are also reported in some carcinosarcomas. It is possible that the known genetic defect on chromosome 9 in this patient contributed to the development of carcinosarcoma. Bhattacharjee P, Leffell D, McNiff JM. Primary cutaneous carcinosarcoma arising in a patient with nevoid basal cell carcinoma syndrome.

Primary cutaneous carcinosarcoma: review and presentation of a case with emphasis on clinical characteristics and management

Carcinosarcomas are rare malignant biphasic tumours that contain intermingled carcinomatous and sarcomatous elements. Primary cutaneous carcinosarcomas (PCCS) are extremely rare. We present the 20th reported case and review the literature especially regarding histogenesis and clinical features such as diagnosis and management. PCCS resembles other non-melanocytic neoplasms of the skin. Diagnosis is obtained by primary excision with subsequent histologic and immunohistochemical examination. PCCS is a potentially lethal neoplasm, but radical surgery is successful in most cases.