Low-grade Malignancy Research Articles

Radiation Therapy Before Repeat Wide Resection for Unplanned Surgery of Soft Tissue Sarcoma (“Oops” Operation) Results in Improved Disease-Free Survival

PurposeThe main goal of treatment of soft-tissue sarcomas is achieving wide negative margins to improve local control and prevent recurrence. The role of radiation therapy (RT) is well established in sarcomas of the extremities; however, its role in unplanned surgery of soft-tissue sarcoma (when a mass presumed to be benign is resected and the pathology comes back as sarcoma, usually referred to as an “oops” operation) is inconclusive. This article reports on the effect of RT after an unplanned surgery before the reresection. Methods and MaterialsA total of 65 patients who had undergone an unplanned resection of a postoperatively diagnosed soft-tissue sarcoma were treated with RT and/or surgery and retrospectively evaluated for disease progression. Treatment started with RT in 49 cases (75.4%), including 8 cases of no further surgery. A repeat wide resection was performed directly after the initial surgery in 16 patients, followed by RT in 15 of them. ResultsThe disease recurred in 7 out of 49 patients (14.3%) who received RT first and in 9 out of 16 (56.25%) who underwent reoperation before RT (P = .001). Disease-free progression was higher in cases of low-grade malignancy (P = .049). A clinical diagnosis of lipoma was associated with a better outcome than a diagnosis of nonlipoma (P = .034). The presence of residual tumor at reoperation did not affect disease control. Patient age, time between symptom onset and diagnosis, hospital level of initial diagnosis (tertiary versus nontertiary), anatomic site, tumor size, and margin status at the initial excisional biopsy were not significantly correlated with the outcome. ConclusionInitiating treatment with RT followed by unplanned “oops” resection of soft-tissue sarcoma before the reresection improved disease-free survival as opposed to vice versa.

Risk of Malignancy Using the Diagnostic Categories Proposed by the World Health Organization International System for Reporting Pancreaticobiliary Cytopathology

Background: The World Health Organization (WHO) proposed an updated reporting system for pancreaticobiliary cytology, which moves low-grade malignancies to “positive for malignancy” group and serous cystadenoma to “negative for malignancy” group. The WHO system also created two new categories, namely, pancreatic neoplasia-low grade (PaN-Low) and pancreatic neoplasia-high grade (PaN-High), which includes neoplastic mucinous cysts and stratifies them according to their cytologic atypia. The risk of malignancy (ROM) of the new categories of the WHO system needs to be defined. Methods: Cytologic slides of all patients, who underwent endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) biopsy at our institution from January 2010 to December 2021 and had a histopathological or clinical follow-up of at least 6 months, were reviewed and reclassified under the Papanicolaou Society of Cytopathology (PSC) and WHO reporting systems. The absolute ROM was calculated for each category of both reporting systems. Results: A total of 420 EUS-FNA samples from 410 patients were reviewed and reclassified. The absolute ROM for the proposed WHO system was 35% for “nondiagnostic,” 1.0% for “negative for malignancy,” 69.0% for “atypical,” 11% for “PaN-Low,” 100% for “PaN-High,” 91% for “suspicious for malignancy,” and 100% for “malignant.” Comparatively, the absolute ROM under the PSC reporting system was 34% for “nondiagnostic,” 1.0% for negative (for malignancy), 50.0% for “atypical,” 0.0% for “neoplastic: benign,” 16% for “neoplastic: other,” 88% for “suspicious for malignancy,” and 100% for “positive or malignant.” Conclusion: The proposed WHO international reporting system has advantages regarding risk stratification improvement and case management.

Ocular surface squamous neoplasia: An overview

Ocular surface squamous neoplasia (OSSN) is a diverse range of neoplasm arising from squamous epithelium of conjunctiva, limbus and cornea. OSSN is considered as a low grade malignancy but may be locally invasive and require exenteration. It has relatively high recurrence rate. It has multifactorial etiology and specific pathogenesis of lesion has yet to be attributed. Histopathology with immunohistochemistry is a gold standard diagnostic tool.This mini review highlights the pathogenesis, risk factors, various clinical manifestations, latest diagnostic tools and recent development in treatment of OSSN.

ONLY THE INQUIRING MIND SOLVES PROBLEMS

<h3>Objective</h3> Hyalinizing clear cell carcinoma (HCCC) is a rare low-grade malignancy involving mainly the minor salivary glands of the palate. It usually presents as a painless swelling and follows an indolent behavior. Histologically, it demonstrates nests of monomorphic clear cells and cells with eosinophilic granular cytoplasm. The histologic overlap between different tumor types poses diagnostic challenges, and molecular changes are becoming pivotal for classification. Most HCCCs have a t(12;22)(q13;q12) chromosomal translocation, leading to fusion of the EWSR1 and ATF1 genes. The integration of histology, fluorescence in situ hybridization (FISH), and immunohistochemistry confirms the diagnosis of HCCC, dictating the prognosis and management. Most HCCCs have a good outcome and respond well irrespective of treatment. <h3>Case Summary</h3> A 61-year-old man with a medical history significant for hyperlipidemia and diabetes mellitus presented for virtual (video) evaluation of throat pain when swallowing present for 1 month. Limited evaluation exhibited an oral mass and the patient was evaluated in person the next day. Examination revealed a soft, crater-like mass of 3 cm in diameter involving the soft and hard palate. Incisional biopsy demonstrated an infiltrative tumor composed of nests of monotonous, round to polygonal epithelial cells with variably eosinophilic to clear cytoplasm surrounded by hyalinized stroma and with perineural invasion. Immunohistochemistry demonstrated p40 positivity of neoplastic nonductal cells; FISH analysis was positive for EWSR1 rearrangement in 82% of the nuclei. The integrated pathologic findings confirmed the diagnosis of HCCC. Imaging was negative for involved cervical nodes or distant findings. The patient underwent partial maxillectomy and bilateral neck dissection followed by adjuvant radiation therapy. <h3>Conclusions</h3> HCCC is a rare low-grade salivary gland malignancy with indolent behavior. Morphologic features remain the cornerstone of salivary gland tumor assessment; however, ancillary testing now helps define the diagnosis. FISH for EWSR1 is a paramount marker for several tumors; nonetheless, EWRS1 rearrangement is not specific for 1 entity and FISH interpretation should be intertwined with clinical, morphologic, and immunohistochemical features to confirm the diagnosis, predict good prognosis, and guide subsequent management of HCCC. Although virtual care can be an effective triage tool, there must be a low threshold for subsequent in-person evaluation to provide adequate care.

Intraosseous angiolipoma of the mandible: case report of a rare entity

<h3>Background</h3> Intraosseous angiolipoma is a benign adipocytic lesion that may occur at any bony site, with predilec- tion for the long bones, ribs, vertebrae, and the head and neck region. As the name suggests, the lesion is character- ized by the presence of mature adipocytes associated with increased vascularity. Intraosseous angiolipoma of the mandible is an exceedingly rare condition first described by Polte in 1976. Until the present date, only four cases were documented in the English literature. Case description: We report a case of a 58 year-old woman presenting with an asymptomatic lesion of the mandible. CBCT examination shows a hypodense area extending from the api- cal region of the mandibular first molar to the mid portion of the mandibular ramus, right side. Lesion is localized, multilocular, with scalloped borders and partially corticated. There is thinning of the facial and lingual cortices, with focal areas of expansion and bone discontinuity. The cortices of the inferior alveolar canal are not visual- ized in some portions. No root resorption is identified. The clinical impression was ameloblastoma. Enucleation and curettage was performed. Microscopic examination reveals the presence of mature adipocytes surrounded by fibrous connective tissue septa featuring numerous vascular channels. Scattered chronic inflammatory cells includ- ing mast cells are seen. No cellular atypia or increased mitotic activity are identified. <h3>Conclusion</h3> We present the fifth case of an intraosseous angiolipoma involving the mandible. The lesion has been classified as either a hyper- plastic process or a benign neoplasm. The differential diagnosis often includes vascular lesions, benign odontogenic entities, central giant cell granuloma, or low-grade intraosseous malignancies. Due to the scarcity of case reports, the nature of the lesion as well as its preferred treatment method are still to be determined.

Koincidence bilaterálního Warthinova tumoru příušních žláz a epitelio-myoepiteliálního karcinomu parafaryngu

Epithelial-myoepithelial carcinoma (EMEC) is a very rare low-grade malignancy with an incidence of less than 1% of all salivary gland tumors. In the parapharyngeal space, it can arise either from the deep lobe of the parotid gland or from minor salivary glands of the oropharynx. We present a case of a 70-year-old female patient with progressive dysphagia and a bulging of paratonsillar space. A CT scan revealed a cystic formation in the parapharyngeal space and bilateral inhomogeneous tumors in parotid glands. Preoperative FNAB (fine needle aspiration bio­psy) was undiagnostic. Definitive histologic examination after surgical removal confirmed epithelial-myoepithelial carcinoma in the parapharyngeal space and Warthin’s tumors in both parotid glands. Immunohistochemical tests of the EMEC revealed BRAF, COSM476 a COSM775 and PIK3CA mutations, however, no HRAS mutation was detected. This case report shows a rare clinical and radiological presentation that could resemble benign dia­gnosis. Multiple salivary gland tumors are relatively rare conditions. Warthin’s tumor is the most common tumor with this type of behaviour. The coincidence of bilateral Warthin’s tumor of parotid glands with a parapharyngeal epithelial-myoepithelial carcinoma has not been documented in literature so far. Keywords: epithelial-myoepithelial carcinoma – parapharyngeal space cyst – salivary gland – Warthin’s tumor

Nodal Metastases in Pediatric and Adult Acinic Cell Carcinoma of the Major Salivary Glands.

Acinic cell carcinoma (AciCC) is a rare, usually low-grade salivary malignancy. Evidence on rates of lymph node metastases (LNMs) is limited in pediatric patients and varies significantly (4%-45%) in adults. We set out to determine and compare rates of LNMs in pediatric and adult AciCC and to analyze their impact on survival, using the National Cancer Database. Historical cohort study. National Cancer Database. All AciCCs of the major salivary glands with complete clinical and pathologic nodal staging were selected between 2010 and 2016. Patient demographics, tumor characteristics, treatment, and survival were analyzed. Univariable and multivariable regression were performed to determine factors associated with LNMs and survival. We identified 57 (4.6%) pediatric patients (<18 years) and 1192 (95.4%) adults with AciCC. Clinical LNMs were rare in pediatric patients (n < 10) and adults (n = 88, 7.4%). Occult LNMs were uncommon in pediatric patients (n < 5) and adults (n = 41, 4.6%). Three-year overall survival for pediatric patients was 97.8%. Adults with LNM had worse 3-year overall survival than those without (66.0% vs 96.3%, P < .001). In multivariable regression, high-grade disease (hazard ratio, 10.15 [95% CI, 5.60-18.80]; P < .001) and T3-T4 tumors (hazard ratio, 2.80 [95% CI, 1.56-4.97]; P < .001) were associated with LNM in adult patients. LNMs in AciCC of the major salivary glands are rare in children and adults. However, high-grade and T3-T4 tumors are associated with an increased risk of LNM. LNM is associated with worse survival.

Monoclonal Gammopathy-Related Kidney Diseases.

Monoclonal gammopathies occur secondary to a broad range of clonal B lymphocyte or plasma cell disorders, producing either whole or truncated monoclonal immunoglobulins. The kidneys are often affected by these monoclonal proteins, and, although not mutually exclusive, can involve the glomeruli, tubules, interstitium, and vasculature. The nephrotoxic potential of these monoclonal proteins is dependent on a variety of physicochemical characteristics that are responsible for the diverse clinicopathologic manifestations, including glomerular diseases with organized deposits, glomerular diseases with granular deposits, and other lesions, such as C3 glomerulopathy and thrombotic microangiopathy with unique pathophysiologic features. The diseases that involve primarily the tubulointerstitial and vascular compartments are light chain cast nephropathy, light chain proximal tubulopathy, crystal-storing histiocytosis, and crystalglobulin-induced nephropathy with distinct acute and chronic clinicopathologic features. The diagnosis of a monoclonal gammopathy-related kidney disease is established by identification of an underlying active or more commonly, low-grade hematologic malignancy, serologic evidence of a monoclonal gammopathy when detectable, and most importantly, monoclonal protein-induced pathologic lesions seen in a kidney biopsy, confirming the association with the monoclonal protein. Establishing a diagnosis may be challenging at times, particularly in the absence of an overt hematologic malignancy, with or without monoclonal gammopathy, such as proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Overall, the treatment is directed against the underlying hematologic disorder and the potential source of the monoclonal protein.

A rare collision tumor of ovary – Mucinous cystadenoma with adult granulosa cell tumor

Though collision tumors have been reported earlier like serous cystadenoma and mature cystic teratoma, combination of mucinous cystadenoma and adult granulosa cell tumor is rarely reported in the literature. Collision tumors lack the histological cellular intermingling which is seen in composite tumors. Both involve two morphologically and immunohistochemically distinct neoplasms coexisting within a single organ. Mucinous cystadenoma is a benign cystic surface epithelial tumor of ovary. Granulosa cell tumor(GCT) is a low grade malignancy arising from sex cord stromal cells of ovary and need a close follow up for recurrences which may be late. Here we present a case of 50 year old female who presented with lower abdominal pain. Patient underwent staging laporotomy and ovarian specimen sent for histopathological examination, where it was diagnosed as mucinous cystadenoma coexisting with adult granulosa cell tumor. This case report emphasis upon the fact that multiloculated cyst have to be extensively examined grossly, so as not to miss any solid component which might have a bearing on prognosis of the patient. Here the association of mucinous cystadenoma and granulosa cell tumor need close follow up of patient.

Clinicopathological features of very well-differentiated adenocarcinoma of the stomach

Objective: To investigate the clinicopathological features of very well-differentiated adenocarcinoma (VWDA) of the stomach. Methods: The clinicopathological data of 12 cases of VWDA of the stomach were collected retrospectively at the People's Liberation Army Joint Logistics Support Force 989 Hospital (formerly 152 Hospital), Pingdingshan, China, from January 2013 to May 2021. The histological characteristics and immunophenotypes were observed and analyzed with review of current literature. Results: There were 8 males and 4 females with a median age of 63 years (range 47 to 80 years). The tumor involved in the upper part of the stomach in 6 cases, the middle part in 2 cases, and the lower part in 4 cases. The median diameter of the tumors was 17 mm (range 5-65 mm). The tumor cells were similar to absorbent cells, Paneth cells, foveolar epithelial cells, and goblet cells. The cells were arranged in a single layer, and the nuclei were slightly enlarged and located at the base. The nuclei were fusiform to slightly irregular, with loss of nuclear polarity. Early tubular VWDA was found in 9 cases, and the tumor glands were similar to intestinal metaplasia. In two cases the tumors infiltrated into the submucosa. The lesions in the mucosa and submucosa showed the glands with cystic expansion, bending, branching, spiky and abortive growth pattern. One case of early papillary tubular VWDA was confined to the mucosal layer and composed of foveolar-type epithelial cells. There were two cases of advanced papillary tubular VWDA, which consisted of foveolar-type epithelial, pyloric glands, or mucinous neck cells and were associated with intra-lymphatic cancer embolus and lymph node metastases. Background mucosal atrophy and intestinal metaplasia were observed in all cases. Immunohistochemical staining showed intestinal type VWDA in 1 case, mixed gastrointestinal type VWDA in 9 cases, and gastric type VWDA in 2 cases. The Ki-67 proliferation index of 8 cases limited to the mucosa was 40%-70%, 2 cases of infiltration into the submucosa and 2 cases of advanced carcinoma was 10%-25%. All the tumors showed a wild type of p53 protein expression pattern and negative HER2. Adenocarcinoma or high-grade dysplasia was diagnosed on preoperative biopsy in 5 cases, and chronic atrophic gastritis with intestinal metaplasia in 7 cases. The median follow-up time was 28 months (range 12-72 months). No recurrence was found in the 10 patients with early cancer. Of the two patients with advanced carcinoma, one patient had lung metastases and the other died. Conclusions: Gastric VWDA is a rare low-grade malignancy with structural features of highly differentiated adenocarcinoma and extremely low cytological atypia. The diagnostic value of structural abnormality is significantly greater than cytological atypia. The invasive growth of irregular glands in the deep mucosa and submucosa is reliable evidence for diagnosis. The diagnosis of intramucosal VWDA is challenging and very difficult in some biopsy specimens.

Scalp Reconstruction Strategy Based on the Etiology of the Scalp Defects.

Scalp defects of various etiologies require distinct reconstruction strategies. Therefore, the authors divided scalp defects into the following categories: scar alopecia, open scalp wound, benign or low-grade malignant tumor, and high-grade malignancy. The authors reviewed the experience with scalp reconstruction of a single center to determine the factors that affect the reconstructive choices.Patients who underwent scalp reconstruction between 2008 and 2020 were retrospectively reviewed. Reconstruction methods were classified according to the etiology of the defect and were compared for each etiology. Accordingly, a reconstruction algorithm for scalp defects was proposed.A total of 180 patients were included in this study, and the reconstruction methods demonstrated significant differences according to etiology (P < 0.05). For scar alopecia and open scalp wounds, reconstruction methods such as direct repair, local flap transfer, and tissue expander placement were used depending on the defect size. Patients with benign or low-grade malignancies mainly underwent reconstruction with local flaps or skin grafts and tissue expanders for covering the defects. Patients with high-grade malignancies underwent reconstruction with free flaps if they were scheduled for preoperative or postoperative radiation therapy.Various factors, suchas the etiology, size, location, and depthofthe defect, should be considered in scalp reconstruction. The defect etiology is an important factor that determines the reconstructive goal. Our algorithm is based on the etiology of defects and is intended to aid physicians in choosing the appropriate treatment for various scalp defects.

A retrospective cohort study of dermatofibrosarcoma protuberans at a large metropolitan academic center

To the Editor: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade dermal mesenchymal malignancy that commonly presents as a slow-growing, asymptomatic nodule.1,2 Although its metastasis is uncommon, the tumor tends to recur locally following incomplete excision.3 The clinicopathologic features associated with poor outcomes include treatment with wide local excision (WLE), compared with that with Mohs micrographic surgery (MMS); positive histologic margins; the presence of a fibrosarcomatous change; age >50 years; a large tumor size (>5 cm); African American ethnicity; male sex; and a location on the head, neck, or extremity rather than on the trunk.

Interference of Known or Suspected Endometriosis in Reporting FDG PET/CT Performed in Another Indication.

IntroductionEndometriosis is a common gynecologic condition that may be visualized on 18F-FDG PET/CT and mimic lesions of malignancy. We analyzed the interference of known or suspected endometriosis in reporting 18F-FDG PET/CT performed in another indication.ResultsThe PET/CT images of 18 women with known (n = 15) or suspected (n = 3) endometriosis were analyzed. Based on clinical follow-up and results of other imaging, biopsy, and/or postsurgical histology, the presence of lesions of endometriosis at the time of 18F-FDG PET/CT was confirmed in 13 of 18 patients (72%). The per-patient positivity rate of 18F-FDG PET/CT was 8/18 (44%; 95% confidence interval, 22%–69%). The patient-based detection rate of 18F-FDG PET/CT in patients with confirmed lesions of endometriosis was 8/13 (62%; confidence interval, 32%–86%). On per-lesion/site basis, 18F-FDG PET/CT detected 11 of 20 sites (55%) of endometriosis. The SUVmax of these lesions/sites ranged between 1.8 and 5.3 (median, 3.8). In 9 of 18 patients (50%), a total of 13 non–endometriosis-related lesions/sites were detected by 18F-FDG PET/CT; their SUVmax ranged between 2.7 and 23 (median, 9.4).ConclusionThe interference of known or suspected endometriosis in reporting 18F-FDG PET/CT performed in another indication was limited but possible and should be kept in mind, even in postmenopausal women, as the oldest patient with 18F-FDG–positive endometriosis was aged 63 years. The lesions of endometriosis showed inconstant 18F-FDG uptake with overlap of SUVmax with low-grade malignancies. In our series, the greatest SUVmax value of lesion of endometriosis was 5.3, somewhat higher than the threshold of 4 previously proposed for identification of malignant transformation of endometriosis.

Adenoid Cystic Carcinoma Bronchus - A Case Report

Primary adenoid cystic carcinoma of lung is an unusual thoracic neoplasm, which is considered as a slow-growing low-grade malignancy. Usually, it is diagnosed at a higher clinical stage and is difficult to resect due to its central location. A 48-year-old female presented with history of breathlessness. On chest Xray and CT-Thorax showed well defined soft tissue lesion in left main bronchus. She underwent left pneumonectomy and subcarinal node dissection. Histopathological examination was consistent with Adenoid Cystic Carcinoma Bronchus.

Myofibroblastic sarcoma of the oral cavity: a diagnostic dilemma and report of two cases

Introduction: Myofibroblastic sarcoma is designated as a low-grade malignancy, commonly affecting the deep soft tissue of the head and neck. Despite being classified as low-grade, myofibroblastic sarcoma with high-grade features have been reported. Observations: Two such cases affecting the oral cavity, which were diagnosed as different entities upon biopsy, were observed. Case 1 presented as multiple, well-circumscribed soft tissue swellings of the tongue and alveolar mucosa and was diagnosed as synovial sarcoma. Case 2 manifested as a large extensive osseous lesion of the maxilla and was diagnosed as an inflammatory myofibroblastic tumour. Conclusion: Myofibroblastic sarcoma with high-grade features remains a diagnostic dilemma due to its overlapping features with other spectra of fibroblastic/myofibroblastic tumours and lack of consensus regarding its classification as a separate entity. Establishing the definitive diagnosis requires adequate tumour sampling and a systematic clinicopathological approach.

Case Report: Surgical Resection of an Aggressive Pancreatic Solid Pseudopapillary Tumor with Local Advance and Hepatic Metastasis

We are presenting a case of surgical resection of a solid pseudopapillary neoplasm (SPN) of the pancreas with multiple hepatic metastatic neoplasms. SPN is a rare neoplasm with low-grade malignancy usually characterized by a tick-encapsulated mass primarily found in the body and tail of the pancreas. However, in our case, the tumor was located in the head of the pancreas with an invasion of the superior mesenteric vein/portal vein (SMV/PV), along with the presence of multiple hepatic metastases with enlarged retroperitoneal lymph nodes were also observed through radiological examination.

A REPORT OF MAXILLARY SINUS ACINIC CELL CARCINOMA

Introduction: Acinic cell carcinoma (ACC) is a rare malignant tumor of the salivary gland. It usually shows in parotid gland, and rarely shows in other site. Case Report: A 33 years old female from East Nusa Tenggara, came to our outpatient clinic with a complaint of nasal obstruction and recurrent epistaxis on her right nose 1 year ago. Biopsy for the tumor was done by the previous Ear-Nose-Throat (ENT)-specialist and resulted suggestive pleomorphic adenoma. During her visit, we found a red colored mass occupying the right nasal cavity, emerges from the middle meatus. Patient had not received medical nor non-medical therapy after the previously done biopsy, and declined of having any epistaxis episodes during the time. Nasal obstruction was her current complain and what brings her to seek medical care. We continued with a midfacial degloving surgery and removed the tumor, which aroused from maxillary sinus. Histopathology of the tumor shows an acinic cell carcinoma feature. Discussion: We searched all English and Non-English previously ACC reports in nasal cavity to compare with our case and our treatment plan. Conclusion: Sinonasal ACC are known as a rare, slow-growing and low-grade malignancy tumor with an excellent outcome, despite indistinct symptoms.

NON SURGICAL TREATMENT OF CARCINOID LUNGS TUMORS

Typical carcinoid lungs tumors are neuroendocrine bronchopulmonary tumors with a low-grade malignancy, and an atypical carcinoid is an intermediate form of these tumors. Their systemic treatment is greatly influenced by therapeutic evidence derived from the more frequent gastroenteropancreatic neuroendocrine neoplasms. Currently, systemic therapies for lung carcinoids, aiming at controlling tumor growth include long acting somatostatin analogues (SSAs), peptide receptor radionuclide therapy, chemotherapy and molecular-targeted therapy.

Prognostic factors in mammary analogue secretory carcinomas of the parotid gland: Systematic review and meta-analysis.

Mammary analogue secretory carcinomas (MASCs) of the parotid gland are considered as low-grade malignancies with good clinical outcome but lacking data regarding prognostic factors. We performed meta-analysis assessing prognostic factors for disease-free survival (DFS) and overall survival (OS) in 256 patients with MASCs of the parotid gland. A total of 73 studies have met the inclusion criteria and 76.3% of patients were seen with T1 and T2 tumors and negative neck nodes. Lymph node metastasis (57.4%) and distant recurrences (46.2%) were particularly found in T4 tumors (p < 0.001). DFS at 5 and 10 years was 77.9% and 47.2% compared to 88.1% and 77.2% for OS at the same time points. Male sex, T3-T4 tumors, and recurrent disease represented independent worse prognosticators for survival outcome. Altogether, parotid gland MASCs show good long-term outcome, but T4 tumors behave significantly more aggressive and require extended treatment strategies along with close follow-ups.

Darier and Ferrand's Dermatofibrosarcoma with Craniofacial Location: A Rare Entity

Introdcuction: Darier and Ferrand's dermatofibrosarcoma is a rare but not exceptional malignant mesenchymentous skin tumor. It represents 0.1% of malignant skin tumors. First described by Jean Darier and Marcel Ferrant in 1924, it is a slow-growing tumor, currently considered to be a true low-grade malignancy fibrosarcoma. The authors report in this work the clinical and evolutionary aspects of three cases of Darier and Ferrand dermatofibrosarcoma located at the cephalic end. Observations: These were 3 cases of Darier and Ferrand dermatofibrosarcoma encountered between 2010 and 2016. The subjects were female. The ages ranged from 30 to 44 years old. The diagnostic delay varied between 6 and 10 years. The general condition was altered in two cases. The localization was facial in two cases and the scalp in one case. The size of the tumors varied between 15 and 20 cm. All patients underwent maxillofacial computed tomography (CT) and laboratory workup. The clinically suggested diagnosis was confirmed by histological examination. One patient underwent surgical excision with a 3 cm margin. Covering the loss of substance appealed to thin skin grafting. The evolution was favorable after a 7-year follow-up. In the other two cases, the evolution was marked by death before surgical management. Conclusion: DFS is a rare malignant skin tumor with slow local progression that can often be life-threatening. Early diagnosis ensures efficient treatment.