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Rothmund−Thomson Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Vikram K. Mahajan, Dhaarna Wadhwa
RTS patients are prone to develop at least one neoplasm, have a second malignancy, or develop multiple primary cancers [13,33]. However, the overall prevalence of cancers in adults with RTS is unknown. Skin cancers and both isolated and multicentric osteosarcoma are the most common malignancies in RTS, with an estimated prevalence of 5% and 30%, respectively [13]. RTS-associated osteosarcoma has an earlier onset at the mean age of 14 years compared to 17 years in sporadic cases in general population, but with no difference in the site of tumor development (mostly in femur, tibia) and the histological (mostly osteoblastic) subtype [1,13,33]. However, the proportion of 17.9%–25.6% for RTS-associated multicentric osteosarcoma is higher compared to 0.4%–10% for all sporadic cases, suggesting an increased risk for multicentric osteosarcoma [1,34]. RTS patients with pathogenic variants in RECQL4 notably have increased risk for developing osteosarcoma [3]. Malignant fibrous histiocytoma is another mesenchymal tumor reported occasionally [35]. Skin cancers such as basal cell carcinoma, squamous cell carcinoma, Bowen's disease, malignant eccrine poroma, and melanoma occur with an estimated prevalence of 5% and early onset at the mean age of 34 years in individuals with RTS compared to the general population but later than osteosarcoma [13,34,36,37]. These cancers involve non-sun-exposed surfaces, and squamous cell carcinoma is the most common epithelial tumor.
Fibrous tumors
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Histiocytoma is one of the most common benign tumors of the skin and usually does not pose problems in making the correct diagnosis. However, valuable distinctive markers for the differentiation of histiocytoma from dermatofibrosarcoma are factor XIIIa, CD68, and stromelysin 3 with the latter displaying a sensitivity of 100% and specificity of 94%, the former of 100% and 71%. In contrast, CD34 has a sensitivity of 94% for dermatofibrosarcoma protuberans and a specificity of 83%.3,4 Dermatofibrosarcoma protuberans shows positivity for collagen triple helix repeat containing-1 whereas histiocytoma is negative.6
Selected Heritable Skin Diseases of Domesticated Animals
Published in John P. Sundberg, Handbook of Mouse Mutations with Skin and Hair Abnormalities, 2020
Robert W. Dunstan, Robert A. Kennis
Comments — The histiocytic proliferative diseases remain arguably the most confusing and least understood disorders in human dermatology.61–63 Many different diseases are recognized under this heading, with forms that are benign and undergo spontaneous regression to those which are overtly malignant. Canine histiocytic proliferative diseases also have marked variability in behavior. A benign, self-limiting proliferation of histiocytes, known as canine cutaneous histiocytoma, is the most frequently diagnosed disease in young dogs. Although more aggressive putatively histiocytic diseases are recognized infrequently in most canine breeds, only the histiocytic proliferative diseases of the Bernese Mountain dog have a confirmed familial basis. Whether or not the histiocytic cells are Langerhans cell-derived remains to be defined. This may at first seem a matter which could be easily resolved with electron microscopy; however, Birbeck granules, the ultrastructural hallmark of Langerhans cells in humans and laboratory rodents are seldom identified in cells believed to be canine Langerhans cells (dendritic cells devoid of melanosomes situated in the mid-spinous regions). In addition, the use of standard histochemical techniques to identify Langerhans cells in humans and laboratory rodents, such as ATPase staining or immunohistochemical staining for S-100, do not define these cells in dogs (Moore, personal communication). The inability to confirm or deny that these cells are Langerhans cell-derived precludes characterization of these diseases as either an X- or a non-X histiocytosis. Recently, Langerhans cell-specific antibodies have been developed; however, the literature lacks a report of their application to these diseases.
Single piece fronto-temporo-orbito-zygomatic craniotomy: a personal experience and review of surgical technique
Published in British Journal of Neurosurgery, 2018
Manish Sharma, Sridhar Shastri
Third case of spheno-petro-clival meningioma required a second surgery through different approach as tumour extended below the middle third of clivus. Similar wide exposure was obtained in the case of solitary Fibrous histiocytoma where complete gross excision could be achieved. This patient preoperatively had III/V/VII and Lower cranial nerves involvement. A near total tumour excision was achieved without any added deficits (Figure 2). The function of lower cranial nerve recovered over next 6 months. There were two deaths out of 11 cases. The case of giant pituitary adenoma was taken up for surgery in a very poor general condition as a life saving effort. He succumbed to post op sepsis. One of the two cases of aneurysms was readmitted within a month of surgery with pneumonitis and succumbed to septicaemia. She had recovered well from surgery and was ambulant without support for about 20 day post-operatively when she started to worsen and succumbed to pneumonitis and sepsis. None of the cases were found to have any brain contusions/swelling in the post op scans. It was attributed to significantly less use of intra operative retractors.
Conjunctival and Corneal Fibrous Histiocytoma: Brief review
Published in Seminars in Ophthalmology, 2021
Fibrous histiocytoma (FH) encompasses a heterogeneous group of soft tissue tumors that are composed of cells that resemble fibroblasts and histiocytes, arranged in a cartwheel or storiform pattern.1 The tumour is mesenchymal in origin and most commonly occurs in the dermis and superficial subcutis, with rare deeper involvement. Fibrous histiocytoma has both benign and malignant presentations, with benign being more common. Ocular involvement of FH has been well documented in literature with orbit being the most common site.2,3 Rarely though, FH in both benign and malignant forms, have been reported at the corneo-scleral limbus and the conjunctiva.4,5
Ultrastructure of colorectal adenocarcinoma and peritumoral tissue in untreated patients
Published in Ultrastructural Pathology, 2018
Hector L. Osorio, Hector J. Finol, L. Roschman Gonzalez, Carlos E. Sardiñas
Finol et al. (1994) reported very similar alterations to the ones observed by our team in nervous and muscular tissue adjacent to a fibrous histiocytoma. They proposed that one possible explanation for the presence of peritumoral anomalies could be a local manifestation of the “paraneoplastic phenomenon,” a capacity of malign tumors to cause damage in non-invaded organs. Our findings to this point seem to agree with theirs.38