Desmoplastic Research Articles

Odontogenic Fibroma of Maxilla: A Rare Clinical Entity

The odontogenic fibroma (OF) is a rare benign tumour of mesodermal origin characterized by varying amounts of inactive looking odontogenic epithelium embedded in a mature, fibrous stroma. The lesion appears frequently involving the anterior region, when fibroma occurs in the maxilla, whereas they tends to be located in the posterior area, involving the premolar and molar areas, when fibroma occurs in the mandible. We are presenting a case of 36 year old female patient who had cheek swelling and facial disfigurement for 1 year. Biopsy was suggestive of odontogenic tumor. Complete excision was done transorally with sublabial approach and was histopathologically called out as benign odontogenic tumour consistent with an Odontogenic Fibroma. Patient was asymptomatic till 6 months of followup. Desmoplastic fibroma and intra-osseous fibrogenic myxoma should be considered in the differential diagnosis. The tumour should be managed conservatively with enucleation and curettage.

Microvascular reconstruction with fibula flap: case report

Patient male of 10-year-old native of the city of Puno, arriving at the service of head and neck surgery from the National Institute of health of the child to present an expansive left mandibular tumor of 2 years of evolution of insidious onset and progressive course. Compatible with Desmoplastic fibroma diagnosis. Resection of mandibular tumor, up to 10 cm in length, was practiced the hemimandibulectomy since the region right up to the preangular area of the jaw. During this surgical procedure the flap of the fibula, was conducted on the same side as the hemimandibulectomy. Once the fibula osteotomy was performed to rebuild the receptor bed with unilock reconstruction plates and was fixed with screws. Finally the anastomosis was carried out. One of the greatest challenges of the reconstructor surgeon is the coverage of complex defects in extremities, with exhibition of deep tissues and sometimes with absence of them. As described in the literature, the closure of fibula flap is the

A Gigantic Desmoplastic Fibroma Resembling A Malignant Neoplasm: An Intriguing Case Report

Desmoplastic fibromas of bone have been described as a rare non metastasizing locally aggressive benign lesions that histologically resemble a desmoid tumour of the soft tissue Since his original description a number of case reports have been published as many as They are very common in mandible but here we report a fascinating case in a year old young boy in the body of mandible which has grown to gigantic proportion in a short period of time and showed very aggressive radiographic presentation unlike conventional cases In the present case we have described clinical radiological and histopathological features of Desmoplastic fibroma in detail The gigantic size and aggressiveness of the lesion are the highlights in this case report

CLINICAL AND Х-RAY PATTERNSOF DESMOPLASTIC FIBROMAOF MANDIBULAR BONE

This article presents the results of the clinical examination of the patient at the age of 41, who has been hospitalized according to the principle of OMC to the department of oral surgery and multi- specialized hospital of stomatology with a rare bone disease which affects the lower jaw -desmoplastic fibroma. The surveillance has been conducted for three years. Clinical characteristics and Хray patterns of the disease were revealed and the results of instrumental and histological studies were shown.

Desmoplastic Fibroma of the Mandible

Desmoplastic Fibroma of the Mandible

Experience in the treatment of desmoplastic infantile astrocytoma/ganglioglioma

Objective To explore the clinical characteristics, treatment and prognosis of desmoplastic infantile astrocytoma/ganglioglioma(DIA/DIG). Methods A retrospective analysis was conducted from April 2007 to October 2015 on a total of 6 cases with DIG/DIA who were admitted to Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University. Among them, 4 cases were male and 2 were female, and they were aged 2-19 months. The typical imaging findings included nodular enhancement commonly presenting cystic changes in superficial areas of cerebral hemispheres. The size of tumor was relatively large and could involve multiple lobes, in particular, the frontal and parietal lobes. Pathological examination revealed two-way differentiation of tumor cells to glial cells and neuroepithelium with GFAP (+ ), S-100 (+ ) and Vimentin (+ ), and Ki-67 (+ ) proliferation index was relatively low. Tumor resection was performed in all 6 cases. Postoperative subdural effusion and shunting were conducted in 1 case, and 2 cases underwent postoperative subdural effusion. The follow-up time ranged from 1 to 9 years. Results All 6 children with DIA/DIG (grade WHO Ⅰ) underwent total resection of tumors. Among them, 3 cases without undergoing subdural effusion or shunting surgery achieved good outcomes. Except temporary decrease in muscle strength occurring in 1 case, no other neurological disorders or epileptic seizures were reported. Among the 3 cases undergoing subsequent subdural effusion or shunting surgery, 1 case had good outcome, 1 case developed neurologic dysfunction, and 1 case died of infection. No metastasis spread through cerebrospinal fluid or tumor recurrence was observed. Conclusions DIG/DIA is a rare kind of intracranial tumor with common onset at less than 2 years old. The tumors are usually large and located in the superficial areas of the frontal and parietal lobes. DIG/DIA could have its unique imaging characteristics and the diagnosis could be established based on its typical pathological presentation. Good outcomes could be achieved through total resection of tumors. Key words: Glioma; Disease attributes; Therapy; Desmoplastic infantile astrocytoma/ganglioglioma

DESMOPLASTIC FIBROMA OF THE MANDIBLE: A CASE REPORT

Desmoplastic fibroma (DF) is a benign but locally aggressive neoplasm of the bones and it is very rare in the mandible similar to some other intraoral tumors. The aim of this paper is to report the case of a patient with DF of the mandible. A 33-year-old female patient presented a 1 year and 5 months history of pain related to the 48 tooth and increased volume in the region. The physical examination and radiologic findings suggested a single primary lesion. Excisional biopsy was performed, followed by histopathologic examination that revealed the presence of DF. The patient is under observation for a year without signs of recurrence. It is important that dentists be aware of this condition, despite it being rare.

Clinicopathologic analysis of four cases of desmoplastic melanocytic nevus

Objective To investigate pathological characteristics and differential diagnosis of desmoplastic melanocytic nevus. Methods Four cases of desmoplastic melanocytic nevus were analyzed based on the clinical manifestations and histological, immunohistochemical and fluorescence in situ hybridization (FISH) features. Results Of the 4 cases, 2 were male and 2 were female. Their age ranged from 19 to 30 years with the average age being 26.5 years. The skin lesions were located on the extremities in 3 cases, on the vulva in 1 case. Histologically, the lesions were bilaterally symmetrical intradermal nevus. Nevus cells appeared epithelioid and/or fusiform, some were clustered or scattered in the proliferative fibrous tissue. None of lymphocyte aggregation, necrosis or ulceration was observed. Immunohistochemical examination showed positive staining for S100 and Melan A in 3 cases, positive staining for P16 in 2 cases, Ki-67-labeling index less than 5% in all the 4 cases, and negative staining for factor XIII (FXIII) and CD34 in 2 cases. FISH assay showed no copy-number variations in gene loci 6p25 (RREB1) , 6q23 (MYB) , 6p11.1-q11.1 (Cep6) and 11q13 (CCND1) in desmoplastic melanocytic nevus. Conclusion Desmoplastic melanocytic nevus is a kind of histologically unique, benign melanocytic nevus, and immunohistochemical staining for Ki-67, S-100, Melan A and FⅩⅢand FISH assay on melanoma can be helpful for the differential diagnosis between cutaneous fibrous histiocytoma and melanoma. Key words: Nevus, pigmented; Pathologic processes; Skin manifestations; Immunohistochemistry; Desmoplastic melanocytic nevi; FISH

Desmoplastic fibroma versus soft-tissue desmoid tumour of forearm: a case series of diagnosis, surgical approach, and outcome

IV.

Everolimus as a Therapy of a Benign, Infiltrative Growing Bone Tumor of the Mandibula: A Rare Manifestation with Tuberous Sclerosis

We report a patient with tuberous sclerosis (TSC) who shows a firm swelling of the right mandibula (29 × 56 × 45 mm) at the age of 2.6 years. The histologic evaluation demonstrated a desmoplastic fibroma, a benign yet aggressive and infiltrative growing bone tumor with high recurrence rates. The swelling led to a loosening of the teeth and an impairment of the mouth function.

Intraductal neoplasms of the bile duct. A new challenge to biliary tract tumor pathology.

Invasive biliary tract carcinomas are usually tubular adenocaricnomas with abundant desmoplastic reactions and frequent ductal and periductal invasion at the time of the diagnosis. Recently, several intraductal neoplasms of the bile duct, particularly at a pre-invasive stage, have been recognized. They include intraductal papillary neoplasm of the bile duct (IPNB), biliary intraepithelial neoplasm (BilIN), and others, such as intraductal tubulopapillary neoplasm (ITPN) of the bile duct. IPNBs are grossly visible predominantly intraductal-growing papillary neoplasms covered by well-differentiated neoplastic epithelium with fine fibrovascular cores in the dilated bile ducts. Regarding their similarities to intraductal papillary mucinous neoplasm of the pancreas (IPMN) of main pancreatic duct type, some IPNBs resemble IPMN ("pancreatic type"), while others are only somewhat similar or variably different from IPMN ("non-pancreatic type"). Some IPNBs develop via a common oncogenic signaling pathway, and others, particularly those of intestinal type, frequently show GNAS mutations, as in IPMN. BilINs are a microscopically recognizable flat or micropapillary pre-invasive neoplasm and are presumed to precede conventional nodular-sclerosing cholangiocarcinomas. ITPN of the bile duct is a rare neoplasm composed of densely packed tubular glands. These three types of neoplasms are not infrequently associated with invasive adenocarcinoma. Pre-invasive intraglandular neoplasms of the peribiliary glands, another epithelial system in the biliary tree, have been also reported. Further characterization of these intraductal and intraglandular neoplasms of the bile duct is needed to overcome devastating invasive biliary tract carcinoma.

Desmoplastic Soft Tissue Tumor- A Case Report

Abstract not availableBangladesh J Child Health 2015; VOL 39 (3) :164-167

Lower Extremity Desmoplastic Malignant Melanoma in Egypt.

Desmoplastic melanoma (DM) is a type of spindle cell melanoma characterized by the absence of pigment. The clinical diagnosis of DM represents a challenge for the practitioner and the pathologists because it can mimic benign or malignant skin tumors and even inflammatory skin disorders. We here discuss a case of a patient presented with multiple nodular lesions of the lower extremity following electrocautary to a lesion in her sole which was misdiagnosed as planter wart. Our clinical diagnosis was Kaposi sarcoma, hypertrophic lichen, or extensive verruca vulgaris. However, histopathological examination showed spindle-shaped cells positive for Melan-A and S100 revealing the diagnosis of DM.

Extracellular matrix components affect cell migration and invasive potential of cultured human pancreatic ductal adenocarcinoma cells

The tumor microenvironment influences cancer cell behavior in relation to tumor progression, as well as cell proliferation and invasion. Pancreatic ductal adenocarcinoma (PDAC) is characterized by an intense desmoplastic reaction and extracellular matrix (ECM) components in the tumor microenvironment are involved in a cross-talk between tumor cells, stromal fibroblasts and ECM components, influencing tumor cell behavior. We aimed at analyzing in vitro the effect of the crosstalk between PDAC cells and the ECM of the microenvironment by culturing PDAC cells on different ECM proteins used as a substrate, in order to better understand the relationship between cancer cell phenotype and the proteins occurring in the desmoplastic tissue. For this purpose, we analyzed some epithelial-to-mesenchymal transition (EMT) markers and the migration and invasive potential in human HPAF-II, HPAC and PL45 PDAC cells cultured on collagen type I (COL), laminin (LAM) and fibronectin (FN). Interestingly, the expression of E-cadherin was not significantly affected, but some differences were revealed by the wound healing assay. In fact, migration of HPAF-II and PL45 cells was decreased on FN and LAM, and increased on COL, compared to control cells grown on plastic (NC). By contrast, HPAC was very rapid and unaffected by the substrate. SDS-zymography showed that COL induced a strong upregulation of MMP-2 activity in HPAF-II and HPAC cells, and of MMP-9 in HPAF-II and PL45 cells, compared to NC. These preliminary results suggest that ECM components could differently affect PDAC migration and invasion, possibly depending on the differentiation grade. The characterization of the mutual effects elicited by the tumor-stroma interplay on the cancer cell will contribute to better understand the influence of the stroma on PDAC cancer cell phenotype, in order to develop new therapeutic strategies.

Desmoplastic Fibroma of the Bone: Report of an Unusual Location with Literature Review

Desmoplastic Fibroma of the Bone: Report of an Unusual Location with Literature Review

下顎前歯部に生じた類腱線維腫の1例

下顎前歯部に生じた類腱線維腫の1例

Obstructive jaundice caused by desmoplastic small round cell tumor:a report of one case

Obstructive jaundice caused by desmoplastic small round cell tumor:a report of one case

Desmoplastic fibroma of the mandible in young children—a case series

Desmoplastic fibromas are rare, benign, but aggressive lesions, affecting predominantly young people, with an affinity for the mandible. Four patients with desmoplastic fibromas of the mandible, seen between 1995 and 2015 with long-term follow-up, were identified. Three were treated by wide mandibular resection and immediate reconstruction with rib grafts, and one was treated with chemotherapy. In the three resected cases, there has been no recurrence and all rib grafts were successfully incorporated. The case treated by chemotherapy has persistence of the tumour, but it is not progressing. Desmoplastic fibromas in young children respond well to wide mandibular resection and immediate reconstruction with rib grafts. Chemotherapy may halt progression.

FOSL1 immunohistochemistry clarifies the distinction between desmoplastic fibroblastoma and fibroma of tendon sheath.

Although desmoplastic fibroblastoma (DFB) and fibroma of tendon sheath (FTS) are well-established entities, they may show overlapping clinicopathological features. In addition, cytogenetic data showing a shared 11q12 rearrangement in a small number of cases suggest a close link between these entities. A recent microarray study revealed up-regulation of FOSL1 mRNA in DFBs with 11q12 rearrangement. The aim of this study was to clarify the relationship between DFB and FTS. We tested 42 cases diagnosed originally as either DFBs or FTSs for interobserver concordance based on the existing histological criteria and correlated the diagnosis with FOSL1 immunohistochemistry. In addition, FOSL1 gene status was determined by chromogenic in-situ hybridization (CISH). Using joint histological evaluation, 41 of 42 tumours were classified unanimously by three pathologists into 25 DFBs and 16 FTSs, whereas only one case received discordant opinions. Immunohistochemically, all DFBs showed diffuse, strong FOSL1 nuclear immunoreactivity (25 of 25, 100%), while none of the FTSs showed such overexpression. None of the selected 42 DFB mimics overexpressed FOSL1. FOSL1 was not rearranged in seven DFBs tested by CISH. We confirm here that DFB and FTS are two distinct entities that can be distinguished using the existing histological criteria. This distinction corresponds perfectly with FOSL1 immunohistochemical expression status, and diffuse strong FOSL1 expression specific to DFBs sharpens the border between the two categories. FOSL1 overexpression in DFB may not be caused directly by FOSL1 gene rearrangement. FOSL1 may also be a diagnostic aid for differentiating DFB from other histological mimics.

Desmoplastic fibroma of the distal tibia: A case report of a minimally invasive histological diagnosis.

Desmoplastic fibroma (DF) is a benign, rare fibroblastic intraosseous neoplasm histologically resembling a desmoid soft tissue tumor. Although classified as benign, DF frequently exhibits an aggressive behavior, has a moderate-to-high recurrence rate, and often causes pathological fractures and extensive bone destruction. This case report presents an incidentally detected DF of the tibia, which was diagnosed using a minimally invasive approach. A 36-year-old African female patient was referred to the Department of Diagnostic Imaging of Arcispedale Santa Maria Nuova-IRCCS (Reggio Emilia, Italy), to be examined by a computed tomography scan on an outpatient basis, after an x-ray examination of the tibia, which was performed after an injury to exclude the presence of a fracture, revealed a hyperlucency of unknown origin. The aim of this study was to discuss the clinical, histological, immunohistochemical and radiographic characteristics of this rare neoplasm, with a focus on image-guided bone biopsy.