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The American Journal of Dermatopathology Dec 2020Desmoplastic melanoma can be difficult to diagnose and on average have a significantly higher T stage at the time of diagnosis compared with conventional melanomas....
Desmoplastic melanoma can be difficult to diagnose and on average have a significantly higher T stage at the time of diagnosis compared with conventional melanomas. Histologically, these tumors typically consist of spindle cells in a fibrous matrix. The spindle cells may display fibroblast and/or Schwann cell-like features. In this study, we describe the features of 12 cases of desmoplastic melanoma closely simulating neurofibroma. Although the spindle cells in these tumors may be indistinguishable from those of neurofibroma, features such as prominent fibroplasia (12/12), poor lateral circumscription (8/9), diffuse infiltration of subcutaneous tissue (7/9), and lymphoid aggregates (10/12) may be helpful clues to the diagnosis. No immunohistochemical markers were reliable in distinguishing neurofibroma-like desmoplastic melanomas from neurofibroma. Clinical follow-up was available in 8 cases, of which 4 were initially misdiagnosed as benign neoplasms and given no further re-excision. All 4 of these cases recurred; 2 of which showed transformation to a more aggressive phenotype.
Topics: Adult; Aged; Aged, 80 and over; Antigens, Neoplasm; Biomarkers, Tumor; Biopsy; Diagnosis, Differential; Diagnostic Errors; Female; Humans; Illinois; Immunohistochemistry; Intermediate Filaments; Male; Melanoma; Middle Aged; Neoplasm Recurrence, Local; Neurofibroma; New York City; Phenotype; Predictive Value of Tests; Skin Neoplasms; Time Factors; Treatment Outcome; Tumor Suppressor Protein p53
PubMed: 32732692
DOI: 10.1097/DAD.0000000000001754 -
Archives of Pathology & Laboratory... Mar 2006Panfolliculoma is a rare follicular neoplasm with differentiation toward both upper (infundibulum and isthmus) and lower (stem, hair matrix, and bulb) segments of a hair...
Panfolliculoma is a rare follicular neoplasm with differentiation toward both upper (infundibulum and isthmus) and lower (stem, hair matrix, and bulb) segments of a hair follicle. We present an unusual case of cystic panfolliculoma. A 33-year-old Hispanic woman presented with an 8-month history of a 3.0-cm cystic scalp mass. The lesion was excised, and the histologic sections showed a cystic follicular neoplasm that contained corneocytes in basket-woven and laminated array, trichohyalin granules of the inner root sheath, germinative cells, papillae, matrical cells, and "shadow" cells. Cytokeratin 903 and cytokeratin 5/6 immunostains uniformly highlight the tumor cells. Ber-EP4 strongly labels the germinative cells but not the follicular papillae. CD34 labels the surrounding fibrotic stroma and focally the epithelial component.
Topics: Adult; Biomarkers, Tumor; Epidermal Cyst; Female; Follicular Cyst; Hair Follicle; Humans; Keratins; Neoplasms, Basal Cell; Skin; Skin Neoplasms
PubMed: 16519571
DOI: 10.5858/2006-130-389-CP -
European Journal of Medical Research Mar 2009Neurofibromatosis (NF) is one of the most common genetic disorders. Inherited in an autosomal dominant fashion, this phacomatosis is classified into two genetically... (Review)
Review
Neurofibromatosis (NF) is one of the most common genetic disorders. Inherited in an autosomal dominant fashion, this phacomatosis is classified into two genetically distinct subtypes characterized by multiple cutaneous lesions and tumors of the peripheral and central nervous system. Neurofibromatosis type 1 (NF1), also referred to as Recklinghausen's disease, affects about 1 in 3500 individuals and presents with a variety of characteristic abnormalities of the skin and the peripheral nervous system. Neurofibromatosis type 2 (NF2), previously termed central neurofibromatosis, is much more rare occurring in less than 1 in 25 000 individuals. Often first clinical signs of NF2 become apparent in the late teens with a sudden loss of hearing due to the development of bi- or unilateral vestibular schwannomas. In addition NF2 patients may suffer from further nervous tissue tumors such as meningiomas or gliomas. This review summarizes the characteristic features of the two forms of NF and outlines commonalities and distinctions between NF1 and NF2.
Topics: Child; Chromosome Aberrations; Combined Modality Therapy; Female; Genes, Dominant; Humans; Male; Middle Aged; Nervous System Neoplasms; Neurofibroma; Neurofibromatosis 1; Neurofibromatosis 2
PubMed: 19380279
DOI: 10.1186/2047-783x-14-3-102 -
The Israel Medical Association Journal... Jan 2020Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other...
BACKGROUND
Primary retroperitoneal neoplasms (PRN) arise from diverse retroperitoneal tissues. Soft tissue sarcomas (STS) comprise the majority and are well studied. Other non-sarcomatous PRN are very rare and less familiar.
OBJECTIVES
To evaluate the clinicopathologic and radiologic features of non-sarcomatous PRN, as well as the outcome of complete tumor resection (TR).
METHODS
Retrospective data were collected on consecutive patients (June 2006 to January 2015) who underwent resection of retroperitoneal lesions at our department. Final pathology of non-sarcomatous PRN was included.
RESULTS
The study population included 36 patients (26% with PRN). PRN were neurogenic (17%), fat-containing (3%), and cystic (6%). The preoperative diagnosis was correct in only 28%. All patients underwent TR via laparotomy (72%) or laparoscopy (28%), for mean operative time of 120 ± 46 minutes. En bloc organ resection was performed in 11%. Complete TR was achieved in 97%. Intra-operative spillage occurred in 8%. Intra-operative, 90-day postoperative complications, and mortality rates were 11%, 36%, and 0%, respectively. The mean length of stay was 6.5 ± 5.5 days. The median overall survival was 53 ± 4.9 months.
CONCLUSIONS
Familiarity with radiologic characteristics of PRN is important for appropriate management. Counter to STS, other PRN are mostly benign and have an indolent course. Radical surgery is not required, as complete TR confers good prognosis. Expectant management is reserved for small, asymptomatic, benign neoplasms.
Topics: Aged; Female; Ganglioneuroma; Humans; Lipoma; Male; Middle Aged; Neurilemmoma; Neurofibroma; Paraganglioma; Retroperitoneal Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 31927807
DOI: No ID Found -
Joint Bone Spine Jan 2022Aneurysmal bone cyst (ABC) is a benign, locally aggressive tumour that arises predominantly in long bones and spine. Following the encouraging results of denosumab use... (Review)
Review
BACKGROUND
Aneurysmal bone cyst (ABC) is a benign, locally aggressive tumour that arises predominantly in long bones and spine. Following the encouraging results of denosumab use in Giant Cell Tumors (GCT) and the histological similarities between ABC and GCT, the interest on the role of denosumab in the therapeutic arsenal of the most advanced ABC is growing. The purpose of this literature review is to investigate the current state of knowledge about the use of denosumab in ABCs.
METHODS
A literature research was conducted through PUBMED, COCHRANE and GOOGLE SCHOLAR using the keywords "aneurysmal bone cyst" AND "denosumab". Seventeen articles were included.
RESULTS
A total of 43 cases were reported in the literature. There were 23 males, 20 females. The mean age was 15,9±8,1 year. Pain relief and neurological improvement were rapid and sustained. Radiological assessment showed ossification and/or volume reduction in 36/39 patients. Eight patients (18,6%) presented a recurrence after or during denosumab therapy of whom 7 were adults. Adverse events occurred in 11 patients, 5 of them were admitted to the intensive care unit due to hypercalcemia.
CONCLUSION
Denosumab use in non-surgical ABCs has shown a positive impact in pain and neurological symptoms. The oncological outcome remains unclear with a recurrence rate of 18,6% during/after denosumab therapy, mostly in adults. However, regarding the potential clinical benefits, its use might be discussed in the most advanced cases. Further research and clinical trials are mandatory to precise its belonging in the therapeutic arsenal.
Topics: Adult; Bone Cysts, Aneurysmal; Bone Density Conservation Agents; Bone Neoplasms; Bone and Bones; Denosumab; Female; Humans; Hypercalcemia; Infant, Newborn; Male
PubMed: 34481945
DOI: 10.1016/j.jbspin.2021.105260 -
Veterinary Medicine and Science Sep 2022A 10-year-old castrated male Maltese dog was presented with chronic swelling that had been present for at least 5 years in the medial canthus of the right eye (OD).
BACKGROUND
A 10-year-old castrated male Maltese dog was presented with chronic swelling that had been present for at least 5 years in the medial canthus of the right eye (OD).
OBJECTIVES
To describe the treatment outcome of dacryops with dacryolithiasis.
METHODS
Bilateral patency of the nasolacrimal system was confirmed by flushing of both upper and lower puncta. Ocular ultrasonography revealed a well-defined, oval-shaped, heterogeneous mass with several hyperechoic foci. Dacryocystorhinography revealed no connection between the mass and lacrimal canaliculus. Gentle blunt dissection of the fibrous connective tissue around the cystic mass was performed. The mass was removed, which intraluminally contained multiple calculi.
RESULTS
Histopathologically, the cystic structure was lined by simple cuboidal epithelium and surrounded by smooth muscle actin positive myoepithelial cells consistent with dacryops derived from the lacrimal glandular ductal system. In addition, several spherical basophilic minerals were observed in the lumen, which were identified as dacryoliths.
CONCLUSION
Surgical removal of this dacryops with dacryolithiasis was curative without recurrence after four months.
Topics: Actins; Animals; Cysts; Dog Diseases; Dogs; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Male; Minerals
PubMed: 35667039
DOI: 10.1002/vms3.853 -
Wiadomosci Lekarskie (Warsaw, Poland :... 2021The aim is to give a morphological characteristic of tumors and tumor-like lesions of the salivary glands in the course of a comprehensive analysis of the surgical...
OBJECTIVE
The aim is to give a morphological characteristic of tumors and tumor-like lesions of the salivary glands in the course of a comprehensive analysis of the surgical material.
PATIENTS AND METHODS
Materials and methods: The study used surgical material from 67 patients with various pathologies of the salivary glands. The patients were treated at Kyiv City Clinical Hospital No. 12 (Ukraine, Kiev) from 2014 to 2018. The resulting material was fixed in a 10% solution of neutral formalin (pH 7.4) for 24-48 hours, carried out according to the generally accepted technique and embedded in paraffin. Serial sections 2 μm thick were made from paraffin blocks and stained with hematoxylin and eosin. Microspecimens were studied, using Olympus BX-41 microscope (Japan).
RESULTS
Results: A comprehensive morphological study of the surgical material of patients with salivary gland pathology undergoing treatment at Kyiv City Clinical Hospital No. 12 (Ukraine, Kiev) for the period from 2014 to 2018 revealed benign tumors and tumor-like lesions of these glands. Pleomorphic adenomas and adenolymphomas represented benign tumors of the salivary glands, while cysts represented tumor-like lesions. Different ratios of parenchymal and stromal components characterized pleomorphic adenomas of the salivary glands. On this basis, we identified three tumor variants (mesenchymal (15 cases, 50.0 %); mixed or classic (10 cases, 33.3 %); epithelial (5 cases, 16.7 %)). A characteristic microscopic feature of salivary gland adenolymphomas was a pronounced predominance of the parenchyma over the stroma. A well-defined wall, represented by connective tissue fibers and epithelial lining, characterized the cysts of the salivary glands. The connective tissue capsule separating the tumor tissue from the intact tissue of the salivary gland was clearly pronounced in adenolymphomas, and in pleomorphic adenomas it could be clearly expressed, intact, with tumor invasion or absent. In mesenchymal and mixed variants of pleomorphic adenomas, in comparison with the epithelial variant, there was a frequent tumor invasion of the capsule, thinning of the capsule or its absence.
CONCLUSION
Conclusions: Morphological examination of the surgical material allows us to diagnose tumors (pleomorphic adenomas and adenolymphomas) and non-tumor lesions (cysts) of the salivary glands. This, of course, is of great therapeutic, diagnostic and prognostic value. Among the identified pathology of the salivary glands, pleomorphic adenomas cause certain difficulties in morphological diagnosis due to their structural diversity and heterogeneity, sometimes requiring morphometric, immunohistochemical or genetic research.
Topics: Adenoma, Pleomorphic; Cysts; Humans; Salivary Gland Neoplasms; Salivary Glands; Ukraine
PubMed: 34156006
DOI: No ID Found -
Annual Review of Pathology 2012Neurofibromatosis type 1 (NF1) is a genetic disease that results from either heritable or spontaneous autosomal dominant mutations in the NF1 gene. A second-hit mutation... (Review)
Review
Neurofibromatosis type 1 (NF1) is a genetic disease that results from either heritable or spontaneous autosomal dominant mutations in the NF1 gene. A second-hit mutation precedes the predominant NF1 neoplasms, which include myeloid leukemia, optic glioma, and plexiform neurofibroma. Despite this requisite NF1 loss of heterozygosity in the tumor cell of origin, nontumorigenic cells contribute to both generalized and specific disease manifestations. In mouse models of plexiform neurofibroma formation, Nf1 haploinsufficient mast cells promote inflammation, accelerating tumor formation and growth. These recruited mast cells, hematopoietic effector cells long known to permeate neurofibroma tissue, mediate key mitogenic signals that contribute to vascular ingrowth, collagen deposition, and tumor growth. Thus, the plexiform neurofibroma microenvironment involves a tumor/stromal interaction with the hematopoietic system that depends, at the molecular level, on a stem cell factor/c-kit-mediated signaling axis. These observations parallel findings in other NF1 disease manifestations and are clearly relevant to medical management of these neurofibromas.
Topics: Animals; Antineoplastic Agents; Benzamides; Hematopoietic System; Humans; Imatinib Mesylate; Mast Cells; Neurofibroma, Plexiform; Neurofibromatosis 1; Neurofibromin 1; Piperazines; Proto-Oncogene Proteins c-kit; Pyrimidines; Signal Transduction; Stem Cell Factor; Tumor Microenvironment
PubMed: 22077553
DOI: 10.1146/annurev-pathol-011811-132441 -
Head and Neck Pathology Jun 2022The term 'choristoma' refers to normal appearing tissue in an abnormal location. We describe a case of choristoma with gastric epithelium of the dorsal tongue in a...
The term 'choristoma' refers to normal appearing tissue in an abnormal location. We describe a case of choristoma with gastric epithelium of the dorsal tongue in a pediatric patient. Lingual choristomas are uncommon cystic or solid lesions which may demonstrate different types of tissue (e.g. gastric epithelium, respiratory epithelium, osseous and neuroglial tissue) histologically. Choristomas with gastric epithelium, also known as heterotropic gastric mucosa or foregut duplication cysts, are thought to arise from pluripotential cells of the embryonic foregut. They most frequently involve the anterior two-thirds of the tongue. Most patients are asymptomatic, but larger lesions may lead to feeding and breathing difficulties. Pathologic evaluation and surgical excision remain the mainstay of diagnosis and treatment, respectively. The pathologic characteristics of other congenital tongue lesions are also discussed.
Topics: Child; Choristoma; Cysts; Epithelium; Gastric Mucosa; Humans; Tongue; Tongue Diseases
PubMed: 34259980
DOI: 10.1007/s12105-021-01362-0 -
Indian Journal of Dental Research :... 2019Nasolabial cysts are a rare non-odontogenic, soft-tissue, developmental cyst reported till date in the sublabial area and anterior maxillary region. The cyst is a slowly... (Review)
Review
Nasolabial cysts are a rare non-odontogenic, soft-tissue, developmental cyst reported till date in the sublabial area and anterior maxillary region. The cyst is a slowly enlarging asymptomatic swelling and non-painful. The cyst is believed to be associated with remnants of the nasolabial duct. In this report, we report a nasolabial cyst of a 48-year-old man in whom the cyst occurred in the buccal mucosa. To the best of our knowledge, this is the first case of nasolabial cyst occurring entirely in the buccal mucosa without involving vestibule. The etiopathogenesis of the cyst is reviewed in light of this case.
Topics: Cysts; Edema; Humans; Male; Middle Aged; Mouth Mucosa; Nose Diseases
PubMed: 31939378
DOI: 10.4103/ijdr.IJDR_861_19