-
Journal of Pharmacy & Bioallied Sciences Jul 2023Desmoplastic ameloblastoma (DA) is a rare variant of conventional ameloblastoma. It accounts for only 4%-13% of all ameloblastomas. DA was included in the World Health...
Desmoplastic ameloblastoma (DA) is a rare variant of conventional ameloblastoma. It accounts for only 4%-13% of all ameloblastomas. DA was included in the World Health Organization Classification of Head and Neck Tumors (WHO-2005) as a variant of ameloblastoma with specific clinical, imaging, and histological features. The desmoplastic variant of ameloblastoma usually appears in the anterior and premolar regions as a mixed radiolucent and radiopaque lesion, sometimes resembling a benign fibro-osseous lesion.Ameloblastoma is a locally aggressive tumor that may cause recurrence and in rare cases, malignant transformation with repeated postsurgical recurrences. In this paper, we present a case of a 28-year-old female with swelling in the left upper jaw, a biopsy of which turned out to be DA.
PubMed: 37654261
DOI: 10.4103/jpbs.jpbs_44_23 -
Surgical Pathology Clinics Dec 2021Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap... (Review)
Review
Fibrous and fibro-osseous tumors are some of the most common benign lesions involving bones. Although many of the histomorphologic features of these tumors overlap significantly, an interdisciplinary approach helps to consolidate the classification of these tumors. Herein, the clinical, radiologic, and pathologic features of lesions within these categories are described.
Topics: Bone Neoplasms; Fibroma, Ossifying; Fibrous Dysplasia of Bone; Humans
PubMed: 34742489
DOI: 10.1016/j.path.2021.06.011 -
Cancer Science Sep 2023Desmoplastic reaction is a fibrosis reaction that is characterized by a large amount of dense extracellular matrix (ECM) and dense fibrous stroma. Fibrotic stroma around... (Review)
Review
Desmoplastic reaction is a fibrosis reaction that is characterized by a large amount of dense extracellular matrix (ECM) and dense fibrous stroma. Fibrotic stroma around the tumor has several different components, including myofibroblasts, collagen, and other ECM molecules. This stromal reaction is a natural response to the tissue injury process, and fibrosis formation is a key factor in pancreatic cancer development. The fibrotic stroma of pancreatic cancer is associated with tumor progression, metastasis, and poor prognosis. Reportedly, multiple processes are involved in fibrosis, which is largely associated with the upregulation of various cytokines, chemokines, matrix metalloproteinases, and other growth factors that promote tumor growth and metastasis. Fibrosis is also associated with immunosuppressive cell recruitment, such as regulatory T cells (Tregs) with suppressing function to antitumor immunity. Further, dense fibrosis restricts the flow of nutrients and oxygen to the tumor cells, which can contribute to drug resistance. Furthermore, the dense collagen matrix can act as a physical barrier to block the entry of drugs into the tumor, thereby further contributing to drug resistance. Thus, understanding the mechanism of desmoplastic reaction and fibrosis in pancreatic cancer will open an avenue to innovative medicine and improve the prognosis of patients suffering from this disease.
Topics: Humans; Pancreatic Neoplasms; Pancreas; Extracellular Matrix; Cytokines
PubMed: 37480223
DOI: 10.1111/cas.15890 -
World Neurosurgery Dec 2020The purpose of this study was to investigate clinical, pathological, and prognostic discrepancies between infantile and noninfantile desmoplastic...
OBJECTIVE
The purpose of this study was to investigate clinical, pathological, and prognostic discrepancies between infantile and noninfantile desmoplastic astrocytoma/ganglioglioma patients.
METHODS
From January 2012 to December 2019, we retrospectively reviewed patients aged <18 years who underwent craniotomies at Beijing Tiantan Hospital. Patients diagnosed with desmoplastic infantile astrocytoma and ganglioglioma (DIA/DIG) were included.
RESULTS
The group consisted of 9 infantile patients and 8 noninfantile patients. The mean age of onset was 30.11 months in infantile patients and 103.75 months in noninfantile patients. Comparing with infantile patients, noninfantile patients had a mild female predominance (P = 0.335). The most common presentation in noninfantile patients was seizure (n = 4, 50%), whereas abnormal head circumference (n = 3, 33.3%) was the most common presentation in the infantile group. All cases showed a ki-67 index <2%. Preoperative tumor volume in infantile patients (213.98 cm) was larger than that in noninfantile patients (21.99 cm) (P = 0.043). Gross total resection was achieved in 5 (55.6%) infantile patients and 6 (75%) noninfantile patients (P = 0.62). All patients are alive by last follow-up visit, and 1 infantile patient recurred 8 months postoperative.
CONCLUSIONS
Infantile and noninfantile patients with DIA/DIGs share similar clinical and histopathological features. Compared with infantile patients, noninfantile patients tend to have different symptom predominance. Lesions in noninfantile patients are prone to present with different cystic-solid patterns and smaller volume. Patients with DIA/DIGs have favorable prognosis regardless of extent of resection.
Topics: Age of Onset; Astrocytoma; Brain Neoplasms; Child, Preschool; Female; Ganglioglioma; Head; Humans; Infant; Ki-67 Antigen; Magnetic Resonance Imaging; Male; Margins of Excision; Neoplasm Recurrence, Local; Neurosurgical Procedures; Prognosis; Seizures; Sex Factors; Treatment Outcome
PubMed: 32822952
DOI: 10.1016/j.wneu.2020.08.091 -
World Journal of Gastroenterology Jul 2022Liver is the most common site of metastases of colorectal cancer, and liver metastases present with distinct histopathological growth patterns (HGPs), including... (Review)
Review
Liver is the most common site of metastases of colorectal cancer, and liver metastases present with distinct histopathological growth patterns (HGPs), including desmoplastic, pushing and replacement HGPs and two rare HGPs. HGP is a miniature of tumor-host reaction and reflects tumor biology and pathological features as well as host immune dynamics. Many studies have revealed the association of HGPs with carcinogenesis, angiogenesis, and clinical outcomes and indicates HGP functions as bond between microscopic characteristics and clinical implications. These findings make HGP a candidate marker in risk stratification and guiding treatment decision-making, and a target of imaging observation for patient screening. Of note, it is crucial to determine the underlying mechanism shaping HGP, for instance, immune infiltration and extracellular matrix remodeling in desmoplastic HGP, and aggressive characteristics and special vascularization in replacement HGP (rHGP). We highlight the importance of aggressive features, vascularization, host immune and organ structure in formation of HGP, hence propose a novel "advance under camouflage" hypothesis to explain the formation of rHGP.
Topics: Cell Proliferation; Colorectal Neoplasms; Humans; Liver Neoplasms; Neovascularization, Pathologic
PubMed: 36051338
DOI: 10.3748/wjg.v28.i26.3101 -
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 -
Head and Neck Pathology Mar 2023Desmoplastic melanoma is a rare subtype of melanoma mainly appearing on sun-exposed skin. Clinically, it is many times non-pigmented and therefore the diagnosis is often... (Review)
Review
BACKGROUND
Desmoplastic melanoma is a rare subtype of melanoma mainly appearing on sun-exposed skin. Clinically, it is many times non-pigmented and therefore the diagnosis is often not suspected.
METHODS
Review article.
RESULTS
In this paper we review the main histopathological, immunohistochemical, and molecular features of desmoplastic melanoma, as well as the top 10 morphologic differential diagnoses which should be considered in most cases. The histopathological pattern can be many times deceptive, mimicking a scar, a fibrous reaction, a fibrohistiocytic tumor such as a dermatofibroma, a vascular tumor such as angiosarcoma, a smooth muscle tumor such as leiomyosarcoma, or a neural tumor. Although an overlying atypical junctional melanocytic proliferation may be seen in most cases, it is absent in a significant percentage (up to 30%) of cases, making the diagnosis even more difficult in those instances. The range of diagnostic pitfalls is wide, which may present disastrous prognostic consequences.
CONCLUSION
Desmoplastic melanoma is often a difficult diagnosis to make, as it frequently shows nonspecific clinical findings and overlapping histologic features with many other tumors. However, the potential clinical and prognostic consequences of misdiagnosis as another entity are great. Therefore, this diagnosis must always be kept in mind when encountering spindle cell tumors affecting the head and neck area.
Topics: Humans; Diagnosis, Differential; Skin Neoplasms; Melanoma; Prognosis; Biomarkers, Tumor
PubMed: 36928737
DOI: 10.1007/s12105-023-01536-y -
Acta Cytologica 2022Small round cell tumors (SRCTs) are a broad category of diverse malignant tumors composed of monotonous undifferentiated cells. Involvement of serous fluids by SRCT is... (Review)
Review
BACKGROUND
Small round cell tumors (SRCTs) are a broad category of diverse malignant tumors composed of monotonous undifferentiated cells. Involvement of serous fluids by SRCT is rare; however, the identification of exfoliated malignant cells is a crucial component of management and has significant implications for treatment and prognosis. The most common effusion tumors with SRCT morphology include Ewing sarcoma, synovial sarcoma, rhabdomyosarcoma (RMS), small-cell neuroendocrine carcinoma (SCNC), and desmoplastic SRCT, and the cytomorphologic distinction between these tumors is challenging. The purpose of this article is to describe the morphologic features of the most common SRCT in fluids and propose helpful ancillary testing.
SUMMARY
Effusion SRCTs display similar primitive and undifferentiated morphologic features although each has subtle variations. Ewing sarcoma is a mesenchymal neoplasm and harbors characteristic translocations t(11;22) (EWSR1-FLI1) or t(21;22) (EWSR1-ERG). In fluids, Ewing sarcoma shows poorly differentiated cells of variable size with round to oval nuclei, prominent nucleoli, and scant cytoplasm. In contrast, synovial sarcoma typically involves extremities and expresses a fusion transcript in t(X;18) (SS18-SSX). This soft tissue neoplasm demonstrates uniform cells with irregular nuclear contours, characteristic nuclear folding, and scant cytoplasm. RMS is a neoplasm arising from skeletal muscle, and the alveolar subtype demonstrates a translocation in t(2;13) (PAX3-FOXO1). The malignant cells show a spectrum of small round cells and pleomorphic large cells with rhabdoid morphology. RMS cells characteristically express myogenin and MyoD1, markers of skeletal muscle differentiation. Although SCNC is not a classic SRCT, the morphology is similar. SCNC demonstrates tight clusters of malignant cells with nuclear molding and salt-and-pepper chromatin. This tumor classically has neuroendocrine differentiation and is positive for synaptophysin and chromogranin on immunohistochemistry. And last, desmoplastic SRCT typically presents as an intra-abdominal mass in young men and characteristically harbors the translocation t(11;22) (p13;q12) (EWSR1-WT1). Cytomorphologically, the tumor shows small monomorphic cells occasionally arranged as rosette-like structures.
KEY MESSAGE
The diagnosis of SRCT can be made in effusion samples and is best achieved with a combination of morphologic features, clinical history, and ancillary testing.
Topics: Biomarkers, Tumor; Carcinoma, Small Cell; Diagnosis, Differential; Humans; Immunohistochemistry; Male; Sarcoma; Sarcoma, Ewing; Soft Tissue Neoplasms; Translocation, Genetic
PubMed: 34218227
DOI: 10.1159/000516497 -
Frontiers in Immunology 2022The relationships between tumor stromal features (such as desmoplastic reaction, myxoid stroma, and keloid-like collagen bundles) and immune cells in the colorectal...
BACKGROUND
The relationships between tumor stromal features (such as desmoplastic reaction, myxoid stroma, and keloid-like collagen bundles) and immune cells in the colorectal carcinoma microenvironment have not yet been fully characterized.
METHODS
In 908 tumors with available tissue among 4,465 incident colorectal adenocarcinoma cases in two prospective cohort studies, we examined desmoplastic reaction, myxoid stroma, and keloid-like collagen bundles. We conducted multiplex immunofluorescence for T cells [CD3, CD4, CD8, CD45RO (PTPRC), and FOXP3] and for macrophages [CD68, CD86, IRF5, MAF, and MRC1 (CD206)]. We used the inverse probability weighting method and the 4,465 incident cancer cases to adjust for selection bias.
RESULTS
Immature desmoplastic reaction was associated with lower densities of intraepithelial CD3CD8CD45RO cells [multivariable odds ratio (OR) for the highest (vs. lowest) density category, 0.43; 95% confidence interval (CI), 0.29-0.62; P <0.0001] and stromal M1-like macrophages [the corresponding OR, 0.44; 95% CI, 0.28-0.70; P = 0.0011]. Similar relations were observed for myxoid stroma [intraepithelial CD3CD8CD45RO cells (P <0.0001) and stromal M1-like macrophages (P = 0.0007)] and for keloid-like collagen bundles (P <0.0001 for intraepithelial CD3CD8CD45RO cells). In colorectal cancer-specific survival analyses, multivariable-adjusted hazard ratios (with 95% confidence intervals) were 0.32 (0.23-0.44; P <0.0001) for mature (vs. immature) desmoplastic reaction, 0.25 (0.16-0.39; P <0.0001) for absent (vs. marked) myxoid stroma, and 0.12 (0.05-0.28; P <0.0001) for absent (vs. marked) keloid-like collagen bundles.
CONCLUSIONS
Immature desmoplastic reaction and myxoid stroma were associated with lower densities of tumor intraepithelial memory cytotoxic T cells and stromal M1-like macrophages, likely reflecting interactions between tumor, immune, and stromal cells in the colorectal tumor microenvironment.
Topics: Colorectal Neoplasms; Humans; Keloid; Prognosis; Prospective Studies; Tumor Microenvironment
PubMed: 35392092
DOI: 10.3389/fimmu.2022.840198 -
Journal of Nuclear Medicine : Official... Sep 2023Desmoplastic small round cell tumor (DSRCT) is a rare, radiosensitive, yet difficult-to-treat sarcoma subtype affecting predominantly male adolescents. Extensive...
Desmoplastic small round cell tumor (DSRCT) is a rare, radiosensitive, yet difficult-to-treat sarcoma subtype affecting predominantly male adolescents. Extensive intraperitoneal seeding is common and requires multimodal management. With no standard therapy established, the prognosis remains poor, and new treatment options are needed. We demonstrate the clinical potential of C-X-C motif chemokine receptor 4 (CXCR4)-directed imaging and endoradiotherapy in DSRCT. Eight male patients underwent dual-tracer imaging with [F]FDG and CXCR4-directed [Ga]pentixafor PET/CT. A visual comparison of both tracers, along with uptake quantification in active DSRCT lesions, was performed. [Ga]pentixafor uptake was correlated with immunohistochemical CXCR4 expression on tumor cells. Four patients with end-stage progressive disease underwent CXCR4-based endoradiotherapy. We report the safety, response by RECIST 1.1, and survival after endoradiotherapy. Uptake of [Ga]pentixafor in tumor lesions was demonstrated in all patients with DSRCT, providing diagnostic power comparable to [F]FDG PET. Corresponding CXCR4 expression was confirmed by immunohistochemistry in all DSRCT biopsies. Finally, 4 patients were treated with CXCR4-directed [Y]endoradiotherapy, 3 in a myeloablative dose range with subsequent autologous stem cell transplantation. All 3 required transfusions, and febrile neutropenia occurred in 2 patients (resulting in 1 death). Notably, severe nonhematologic adverse events were absent. We observed signs of response in all 3 patients, translating into disease stabilization in 2 patients for 143 and 176 d, respectively. In the third patient, postmortem autopsy confirmed a partial pathologic response. We validated CXCR4 as a diagnostic biomarker and a promising target for endoradiotherapy in DSRCT, demonstrated its feasibility, and provided the first evidence of its clinical efficacy.
Topics: Adolescent; Humans; Male; Female; Positron Emission Tomography Computed Tomography; Fluorodeoxyglucose F18; Hematopoietic Stem Cell Transplantation; Gallium Radioisotopes; Desmoplastic Small Round Cell Tumor; Transplantation, Autologous; Peptides, Cyclic; Coordination Complexes; Receptors, CXCR4
PubMed: 37348915
DOI: 10.2967/jnumed.123.265464