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The British Journal of Radiology Feb 2012Desmoplastic small round cell tumour (DSRCT) belongs to the histological descriptive category of small round blue cell tumours. DSRCT primarily occurs in adolescents and... (Review)
Review
Desmoplastic small round cell tumour (DSRCT) belongs to the histological descriptive category of small round blue cell tumours. DSRCT primarily occurs in adolescents and young adults between the ages of 15 and 25 years and has a male predominance. DSRCT is an aggressive disease with a poor prognosis; timely diagnosis is therefore critical to the management of these patients. Although their radiographic appearances overlap with other aggressive malignancies, there are certain imaging features that can suggest the diagnosis and expedite the initiation of appropriate therapy. The aim of our pictorial review is to describe the imaging findings of primary and metastatic DSRCT in adults.
Topics: Adult; Contrast Media; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Female; Fluorodeoxyglucose F18; Humans; Magnetic Resonance Imaging; Male; Multimodal Imaging; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed
PubMed: 22128126
DOI: 10.1259/bjr/57186741 -
Asian Journal of Surgery Apr 2024
Topics: Humans; Desmoplastic Small Round Cell Tumor; Biomarkers, Tumor
PubMed: 38296682
DOI: 10.1016/j.asjsur.2024.01.038 -
Bio-protocol Mar 2017We have developed a 3D co-culture system composed of fibroblasts and colorectal cancer cells that enables us to study the desmoplastic reaction. This method also enables...
We have developed a 3D co-culture system composed of fibroblasts and colorectal cancer cells that enables us to study the desmoplastic reaction. This method also enables us to study the influence of the desmoplastic reaction on the migration of colorectal cancer cells through the surrounding stroma. This protocol has been previously published (Coulson- Thomas , 2011 ) and is described here in more detail.
PubMed: 34458502
DOI: 10.21769/BioProtoc.2195 -
International Journal of Clinical and... 2019Desmoplastic melanoma (DM) is considered a variant of melanoma, characterized by a paucicellular proliferation of malignant spindled melanocytes with an abundant...
Desmoplastic melanoma (DM) is considered a variant of melanoma, characterized by a paucicellular proliferation of malignant spindled melanocytes with an abundant collagenous or "desmoplastic" stroma and an intense inflammatory response. As DMs lack pigmentation, their appearances vary and can mimic many benign and malignant conditions, thus presenting a diagnostic challenge. Here, we are presenting one case involving the head and two cases involving fingers. One of our cases distinctively invade nerves and bones. We reviewed the literature for many similar cases. Most cases presented positive staining of S-100 and negative or focal positive staining of Melan-A and Melanoma. So far, the mechanisms of the rare entity have not been clearly recognized. Early accurate diagnosis and complete excision of this tumor is necessary. Some researchers considered BRAF-targeted therapy may be limited to a small number of patients with DM. Advanced DM may respond well to anti-PD-1 monotherapy.
PubMed: 31933775
DOI: No ID Found -
Cureus Jun 2019Objective Desmoplastic melanoma (DM) is a rare variant of invasive malignancy of the skin pigmented cells. We present a comprehensive study reporting on US demographics,...
Objective Desmoplastic melanoma (DM) is a rare variant of invasive malignancy of the skin pigmented cells. We present a comprehensive study reporting on US demographics, disease characteristics, and survival, to contribute to the current knowledge and raise awareness of this rare disease. Materials and methods The demographics of DM patients diagnosed from January 1, 2004, to December 31, 2015, were obtained by querying the National Cancer Database. The characteristics of DM were compared with common malignant melanoma (CMM) using univariate and multivariate regression models. Five-year overall survival (OS) curves were estimated using Kaplan-Meier analyses and the Cox proportional regression model. Results Our query found 5,895 patients diagnosed with DM and 292,939 patients with CMM. DM tended to present at an older age, a more advanced stage, and with a Breslow depth greater than 4 mm at diagnosis (P<.05). The Kaplan-Meier survival analysis demonstrated a five-year OS for DM and CMM of 75% and 76%, respectively, without any statistical difference (P=.07). Cox regression analysis demonstrated that age at diagnosis and comorbidities were independent predictors of five-year OS for DM (P<.001). Conclusions Older age, advanced stage, and higher Breslow depth were found to be independent positive factors associated with DM.
PubMed: 31431837
DOI: 10.7759/cureus.4931 -
Head and Neck Pathology Sep 2022Mucosal melanoma is a rare subtype of melanoma that accounts for 1% of all melanomas. The incidence of nasal mucosal melanomas is 0.3 per million. Desmoplastic melanomas... (Review)
Review
Mucosal melanoma is a rare subtype of melanoma that accounts for 1% of all melanomas. The incidence of nasal mucosal melanomas is 0.3 per million. Desmoplastic melanomas are a subtype of melanoma with a reported incidence of 2.0 per million. Although 50% of desmoplastic melanomas are found in the head and neck region, mucosal desmoplastic melanoma is exceedingly rare. In the present study, we report a case of nasal mucosal desmoplastic melanoma and review the literature. A 79-year-old female presented to an outside otolaryngologist with nasal discomfort accompanied by rhinorrhea and was found to have a nasal vestibule mass. An endonasal incisional biopsy was performed yielding a diagnosis of a SOX-10 positive tumor. The patient was referred to our institution for further management. A blue-tinged lesion was identified at the prior biopsy site, and the mass was resected via an open rhinoplasty approach. Final pathology demonstrated an infiltrative spindle cell neoplasm with immunohistochemical patterns supportive of desmoplastic melanoma arising from the nasal vestibule. Due to positive margins, the patient underwent a re-resection with no tumor identified on the re-resected specimen. To our knowledge, this is the third case of nasal mucosal desmoplastic melanoma. We review the clinicopathologic features and management of this rare entity.
Topics: Aged; Female; Humans; Melanoma; Nasal Mucosa; Nose Neoplasms
PubMed: 35257324
DOI: 10.1007/s12105-022-01429-6 -
Cureus Feb 2022Desmoplastic fibroblastoma is a benign soft tissue tumor of indolent nature. It is more prevalent in males in their fourth to sixth decades of life and typically...
Desmoplastic fibroblastoma is a benign soft tissue tumor of indolent nature. It is more prevalent in males in their fourth to sixth decades of life and typically presents in the upper extremities, feet, and back. Other, uncommon locations have been reported as well, including the oral cavity and retroperitoneum. Histological examination demonstrates bland spindle cells in a dense collagenous stroma. The tumor neither recurs nor metastasizes. In this report, we discuss a case of a female patient who presented with symptoms concerning for intra-abdominal sepsis and was subsequently diagnosed with an intraperitoneal desmoplastic fibroblastoma. There is no evidence that this condition has been previously reported in the literature in the English language. The lesion was excised during laparoscopy and the patient showed no evidence of recurrence on magnetic resonance elastography (MRE) imaging 12 months later.
PubMed: 35165648
DOI: 10.7759/cureus.22134 -
Brain Pathology (Zurich, Switzerland) Apr 2007Among the variants of medulloblastoma in the current WHO classification of nervous system tumors, the desmoplastic variant, which has been reported to constitute 5%-25%...
Among the variants of medulloblastoma in the current WHO classification of nervous system tumors, the desmoplastic variant, which has been reported to constitute 5%-25% of pediatric medulloblastomas, is defined by its nodular collections of neurocytic cells bounded by desmoplastic internodular zones. We have studied the frequency, morphological features and biological behavior of medulloblastomas in two contemporaneous SIOP/UKCCSG trial cohorts of children with medulloblastomas, CNS9102 (n = 315) and CNS9204 (n = 35), focusing on tumors with nodular and desmoplastic phenotypes. In children aged 3-16 years (CNS9102), the nodular/desmoplastic medulloblastoma represented 5% of all tumors, while in infants aged <3 years (CNS9204) this variant represented 57% of medulloblastomas. Using iFISH to detect molecular cytogenetic abnormalities in medulloblastomas with a nodular architecture, we demonstrated distinct genetic profiles in desmoplastic and non-desmoplastic (classic and anaplastic) tumors; in particular, abnormalities of chromosome 17 occurred in the latter, but not the former. Significantly different outcomes were demonstrated for classic, nodular/desmoplastic and large cell/anaplastic medulloblastomas in both cohorts. In conclusion, the nodular/desmoplastic medulloblastoma appears to have clinical, genetic and biological characteristics that set it apart from other variants of this tumor.
Topics: Adolescent; Age Factors; Cerebellar Neoplasms; Child; Child, Preschool; Chromosome Aberrations; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Infant; Male; Medulloblastoma
PubMed: 17388946
DOI: 10.1111/j.1750-3639.2007.00058.x -
Future Oncology (London, England) Feb 2017To evaluate clinical, pathologic and genetic features of desmoplastic melanoma (DM).
AIM
To evaluate clinical, pathologic and genetic features of desmoplastic melanoma (DM).
MATERIALS & METHODS
Analysis of all DM records from 1991 to 2015.
RESULTS
The most common location of DMs was the head and neck (69%); median age and follow-up were 60.5 and 7.3 years, respectively. A familial predisposition for DMs and others malignancies was analyzed. Thin Breslow thickness (<4.5 mm) was associated with an intraepidermal component or a previous lentigo maligna, whereas high Breslow thickness (>4.5 mm) was observed in 'pure' DM.
CONCLUSION
DM could progress from an early phase, characterized by an intraepidermal component, to late phase, characterized by a dermal nodule. This hypothesis correlates with melanoma genetic and NF1 mutation, which could be an early event in the progression of DM.
Topics: Aged; Aged, 80 and over; Female; Genes, BRCA1; Genes, BRCA2; Genetic Predisposition to Disease; Humans; Lymphatic Metastasis; Lymphocytes, Tumor-Infiltrating; Male; Melanoma; Middle Aged; Neoplasm Invasiveness; Neoplasm Staging; Skin Neoplasms
PubMed: 27728982
DOI: 10.2217/fon-2016-0334 -
Journal of Family Medicine and Primary... May 2020Desmoplastic ameloblastoma (DA) is a rare variant of ameloblastoma, accounting for approximately 4 to 13% of ameloblastomas. It is uncommon, aggressive in nature, and...
Desmoplastic ameloblastoma (DA) is a rare variant of ameloblastoma, accounting for approximately 4 to 13% of ameloblastomas. It is uncommon, aggressive in nature, and there are high chances of misdiagnosis. Clinical and radiographical features are similar to fibro-osseous lesions of jaw. We reported a case of 35-year-old male patient of DA.
PubMed: 32754542
DOI: 10.4103/jfmpc.jfmpc_322_20