Atypical Fibroxanthoma: The Washington University Experience.

BACKGROUND: Atypical fibroxanthoma (AFX) is a rare dermal neoplasm typically occurring on sun-exposed skin in the elderly. As AFX remains a diagnosis of exclusion, updated characterization and treatment assessments are necessary to support informed diagnosis and management. OBJECTIVE: Characterization of contemporary AFX and surgical outcomes by Mohs micrographic surgery (MMS) and conventional local excision (LE). METHODS: Retrospective cohort analysis of all cases of AFX at our institution from January 2000 through July 2016. RESULTS: Among 75 cases with median age at diagnosis 73 years, most occurred on the head and neck (68) independent of age. Most treated cases (42) underwent MMS alone, with median tissue removal greater for LE (2.6 cm, 4.5 cm) than MMS (0.6 cm, 1.2 cm). Over a median 26 months of follow-up, 6 recurrences were observed among 50 cases, with metastases in 2 cases. Intent-to-treat recurrence rates were 3.4% for MMS and 25% for LE. One nonrecurrent and 2 recurrent cases received revised diagnoses after initial treatment, yielding a true recurrence rate of 8.5%. CONCLUSION: Despite diagnostic confounding by similar pathologies, surgical treatment of AFX remains effective. Tissue-sparing resection by MMS affords the potential for cosmetic and reconstructive advantage, without compromising recurrence compared with conventional excision.

Associated conditions in patients with multiple dermatofibromas: Case reports and literature review.

Dermatofibromas are benign, fibrohistiocytic, dermal tumors. Solitary dermatofibromas may be incidental findings, whereas multiple dermatofibromas may be associated with systemic conditions or previous therapies. Two women and one man with multiple dermatofibromas and an associated systemic condition, immunosuppression, or both, are described. Nine dermatofibromas developed in a woman with hypothyroidism, optic neuritis, and Arnold Chiari I malformation. Five dermatofibromas developed in a woman with breast cancer who had received several systemic antineoplastic therapies. Eleven dermatofibromas developed in a man with HIV whose systemic therapies included acyclovir, darunavir/cobicistat, dolutegravir, etravirine, and ritonavir. Conditions associated with multiple dermatofibromas include autoimmune diseases, cancer, chromosomal abnormalities, immunodeficiencies, metabolic disturbances, and altered physiologic states such as pregnancy. Medications received by patients with multiple dermatofibromas included immunosuppressive agents, psoriasis therapies, and antineoplastic drugs. Multiple dermatofibromas can be observed in patients with associated medical conditions, systemic therapies, or both. Therefore, in individuals presenting with multiple dermatofibromas, not only evaluation for associated disorders, but also review of prior and current drug therapies, should be considered.

Clinical spectrum of atypical fibroxanthoma and undifferentiated pleomorphic sarcoma in solid organ transplant recipients: a collective experience.

BACKGROUND: Atypical fibroxanthoma (AFX) and undifferentiated pleomorphic sarcoma (UPS) are uncommon, spindle cell cutaneous malignancies. Solid organ transplant recipients (SOTRs) are immunosuppressed and therefore have a higher incidence of cutaneous malignancies. OBJECTIVE: We describe the clinical spectrum of AFX and a more-aggressive, deeper variant, UPS, in SOTRs. MATERIALS AND METHODS: A retrospective chart review of AFX and UPS in SOTRs was implemented. Cases from Vanderbilt University, Emory University, Mayo Clinic-Jacksonville, and University of Rochester were included. A literature search included previously published cases. RESULTS: The average age of SOTRs at time of tumor presentation was younger than typically seen in immunocompetent patients for AFX. Rates of local recurrences and metastases were higher in the SOTRs than is noted in the immunocompetent literature. Rates of recurrence were higher in those treated with excision than in those treated with Mohs micrographic surgery (MMS). CONCLUSION: AFX and UPS may have a greater risk for recurrence, metastases, and mortality in SOTRs, in whom early treatment with MMS may demonstrate certain advantages in terms of minimizing risk of recurrence and metastasis. UPS and recurrent tumors should be staged appropriately and may respond to adjuvant radiation therapy and reduction of immunosuppression. Immunohistochemical evaluation is recommended to exclude other spindle cell tumors.

Case report: Bone tumor of the scapula in a patient undergoing liver transplantation.

BACKGROUND: De novo malignancies are serious complications in the late postoperative period after liver transplantation. The most common de novo tumors are skin malignancies, posttransplantation lymphoproliferative disorder, tumors of the head and neck, and Kaposi's sarcoma. Such posttransplant de novo malignancies are apparently rarely found in bone. CASE DESCRIPTION: We describe a patient with a low-grade, aggressive fibrous histiocytoma of the scapula. The patient had undergone liver transplantation 6 years earlier. En bloc resection of the tumor and limb salvage was performed. At the 2-year followup the patient had no signs of local recurrence or metastatic spread; the patient had a Musculoskeletal Tumor Society (MSTS) score of 87. LITERATURE REVIEW: A literature review suggests the main predisposing factors to such malignancies are immunosuppression and its length of use. According to the literature, tumors apparently are rare in bone after liver transplantation, with no clearly documented cases. However, in the presence of such a finding, our study might be the first clearly documented case study of this kind of bone tumor. CLINICAL RELEVANCE: We describe a patient with a bone tumor after liver transplantation. Our literature review suggests liver transplantation and long-term immunosuppression played a role in this patient's tumor.

Giant dermatofibroma with granular cell changes: side-effect of bee-venom acupuncture?

Dermatofibroma (DF) is a common benign fibrohistiocytic tumour with a predilection for the legs in middle-aged women. Giant DF, a rare clinical variant of DF, is characterized by its unusually large size. Granular cell change is typical of granular cell tumour, but can be observed in diverse cell lineages. Traumatic factors may be involved in the pathogenesis of giant DF and cellular granularity. We describe a 49-year-old Korean man with a giant DF showing granular cell differentiation, which may have been caused in part by multiple treatments with bee-venom acupuncture.

[Down syndrome: another cause of immunosuppression associated with multiple eruptive dermatofibromas?]. / Síndrome de Down: ¿otra causa de inmunosupresión asociada con dermatofibromas eruptivos múltiples?

The term "multiple eruptive dermatofibromas" usually refers to a clinical situation characterized by the development of between five and eight dermatofibromas during a period of up to four months. It is usually linked to immunodeficiency associated conditions as autoimmune disorders, hematologic malignancies, HIV infection, and transplants. We report three patients with Down syndrome. One patient had psoriatic arthritis under treatment with methotrexate, one had Graves-Basedow disease, and one had hypercholesterolemia. All three patients developed multiple eruptive dermatofibromas. We suggest that the immunologic disturbances associated with Down syndrome, together with other underlying conditions present in these patients, could trigger the development of cutaneous lesions.

Lung adenocarcinoma metastasizing to fibrous histiocytoma: A case report.

RATIONALE: Tumor-to-tumor metastasis is an uncommon phenomenon, and the tumor metastatic to mesenchymal tumor is extremely rare. To our knowledge, this is the first case of lung adenocarcinoma metastasizing to fibrous histiocytoma. PATIENT CONCERNS: A 58-year-old Chinese woman was admitted to our hospital with a complaint of progressive enlargement of a mass in the right upper arm without pain, heat (localized warmth), redness, and swelling, for a year. DIAGNOSES: Ultrasound revealed a mass with relative clear boundary in the right upper arm near elbow joint. Uneven echoes, and blood flow signals were showed within the mass. The tumor was well-demarcated from the surrounding tissue a thin fibrous capsule. Scattered enlarged cells with hyperchromatic pleomorphic nuclei were dispersed in an otherwise typical cutaneous fibrous histiocytoma. These atypical cells arranged in poorly glandular structures or irregular epithelioid nest and were demonstrated as metastatic lung adenocarcinoma by immunohistochemical staining. We reviewed the biopsy section and found these 2 sites of adenocarcinoma shared similar histologic morphological features. Therefore, the final diagnosis was lung adenocarcinoma metastasizing to fibrous histiocytoma. INTERVENTIONS: The patient was diagnosed with lung adenocarcinoma by bronchoscopic biopsy 3 months ago and received 4 cycles of NP (Vinoreltraye [NVB] + cis-platinum) chemotherapy program. The patient underwent a total resection of the mass in right upper arm. OUTCOMES: The patient died of multiple organ failure after 10 months since she was diagnosed as lung adenocarcinoma. LESSONS: The accumulation of lipid droplets in fibrous histiocytoma may be a potential reason for modifying pre-metastatic niche, and therefore create a tumor microenvironment suitable for metastasis.

Generation of a TP53-modified porcine cancer model by CRISPR/Cas9-mediated gene modification in porcine zygotes via electroporation.

TP53 (which encodes p53) is one of the most frequently mutated genes in cancers. In this study, we generated TP53-mutant pigs by gene editing via electroporation of the Cas9 protein (GEEP), a process that involves introducing the Cas9 protein and single-guide RNA (sgRNA) targeting exon 3 and intron 4 of TP53 into in vitro-fertilized zygotes. Zygotes modified by the sgRNAs were transferred to recipients, two of which gave birth to a total of 11 piglets. Of those 11 piglets, 9 survived. Molecular genetic analysis confirmed that 6 of 9 live piglets carried mutations in TP53, including 2 piglets with no wild-type (WT) sequences and 4 genetically mosaic piglets with WT sequences. One mosaic piglet had 142 and 151 bp deletions caused by a combination of the two sgRNAs. These piglets were continually monitored for 16 months and three of the genome-edited pigs (50%) exhibited various tumor phenotypes that we presumed were caused by TP53 mutations. Two mutant pigs with no WT sequences developed mandibular osteosarcoma and nephroblastoma. The mosaic pig with a deletion between targeting sites of two sgRNAs exhibited malignant fibrous histiocytoma. Tumor phenotypes of TP53 mosaic mutant pigs have not been previously reported. Our results indicated that the mutations caused by gene editing successfully induced tumor phenotypes in both TP53 mosaic- and bi-allelic mutant pigs.

Malignant fibrous histiocytoma associated with coxofemoral arthrodesis.

The discovery of biomaterials led to their use in the manufacture of implants for biomedical applications. In vivo, no metal or alloy is completely inert. The potential toxicity of some of the metals most frequently employed in the manufacture of orthopedic implants has been reported. Their carcinogenic potential has been evaluated in experimental animal models. However, few reports have discussed the potential development of malignant tumors associated with prosthetic structures in humans. The present study documents a case of intraosseous sarcoma that developed in the vicinity of a metallic prosthesis 43 months after a coxofemoral arthrodesis with metallic pins and screws. With this report the authors seek to contribute to the understanding of the potential toxicity and risks of using metallic implants. Since metallic implants employed in the rehabilitation of osteo-muscular-articular disorders usually remain in the organism for long periods of time, the need to monitor the metallic structures and the adjacent tissues is extremely relevant.

Fibrous Histiocytoma-like Spindle-Cell Proliferation in the Nipple After Body-Piercing.

We report the case of a 19-year-old pregnant woman who presented with a nipple tumor. The lesion consisted in a spindle-cell proliferation with histologic features similar to those of fibrous histiocytoma, with a highly vascularized stroma. Although it showed low mitotic activity, scattered marked atypical cells with prominent nucleoli were identified, thus raising concern about the benign nature of the tumor. Immunohistochemical evaluation revealed that the spindle cells were diffusely positive for vimentin, focally positive for CD68, and negative for all the other tested antibodies. The patient had a total excision of the lesion and she is free of disease after 30 months. To our knowledge this is the first reported case of a lesion of this type in the nipple after body-piercing.

Risk of radiation-related subsequent malignant tumors in survivors of Ewing's sarcoma.

Twenty-four long-term survivors of Ewing's sarcoma were identified as being at risk for a second primary tumor. Among this group of patients followed from 3 to 22 years, 4 new bone tumors were observed, whereas 1.2 x 10(-3) were expected. All new tumors arose in heavily irradiated areas. The risk associated with radiation after 3 years was 7.2 cases/million person-years per rad. The cumulative cancer risk over 10 years for irradiated patients was 35% (SE, 15.1%). Intensive chemotherapy (cyclophosphamide and vincristine administered in five or more courses) seemed to exert an enhancing effect, increasing the rate of development of new tumors.

Malignant fibrous histiocytoma complicating mitral valve replacement.

A 67 year old woman developed a fatal febrile illness 8 years after mitral valve replacement for rheumatic valvular heart disease. The final disease persisted for 1 year and was characterized clinically by weakness, weight loss, congestive heart failure and multiple embolic events to the central nervous system and abdominal organs. The source of the emboli was a tumor, malignant fibrous histiocytoma of the left atrium, originating from the anulus fibrosus around the covered base of the prosthetic valve. This unique case suggests the possibility that chronic exposure to materials, such as Dacron, covering prosthetic valves may induce local malignant tumors.

Postradiation malignant fibrous histiocytoma and osteosarcoma of a patient with high telomerase activities.

BACKGROUND: An extremely rare case of postradiation malignant fibrous histiocytoma (MFH) and osteosarcoma (OS) secondary to radiation therapy for leukemia-related osteolytic lesions is presented. In addition, the telomere biology of these tumors was investigated. CASE REPORT: A 14-year-old boy was diagnosed with acute lymphocytic leukemia. The right tibia was irradiated at a total dose of 60 Gy, and the left tibia was irradiated at a total dose of 40 Gy. The left tibia developed MFH and the right tibia developed OS. RESULTS: Telomere reduction (MFH 70.2, OS 70.0%) and high telomerase activities (MFH 12.1, OS 17.7 TPG) were observed. These results reflect an aggressive feature of postradiation sarcomas. CONCLUSION: Prognosis for patients diagnosed with postradiation sarcoma is poor due to its aggressiveness. However, even if sarcoma occurs after irradiation in more than two fields in a single patient, improvements in prognosis are anticipated with appropriate chemotherapies and wide resection.