THE PROGNOSTIC SIGNIFICANCE OF MYXOID MATRIX IN RETROPERITONEAL WELL-DIFFERENTIATED LIPOSARCOMA

Objective: to assess the influence of the myxoid matrix in retroperitoneal well-differentiated liposarcoma(WDLPS ) on the long-term results of surgical treatment of patients.Material and Methods. The study included 111 patients with primary retroperitoneal WDLPS who underwent radical surgical treatment in Federal State Budgetary Institution «N.N. Blokhin National Medical Research Center of Oncology» of the Ministry of Health of the Russian Federation. Histological slides of all surgical specimens were reviewed by experienced pathologist and reclassified according to criteria of WHO (2013) for histological subtypes of the WDLPS . Patients were divided into groups depending on presence or absence of the myxoid matrix in WDLPS and enrolled in intergroup analysis of overall (OS ) and recurrence-free (RFS) survival.Results. OS was significantly worse in the group of patients with the myxoid matrix in the tumor (p=0.002; log-rank test). The median OS was 142 (95 % CI , 108, 176) months in the group without the myxoid matrix, and 84 (95 % CI , 29, 139) months in the group with the myxoid matrix. The 5-year survival rates were 79 % and 44 % in the groups without myxoid matrix and with myxoid matrix, respectively. RFS was also significantly worse in the group of patients with the myxoid matrix than in the group of patients without the myxoid matrix (p=0.006; log-rank test). The median RFS was 55 (95 % CI , 38, 72) months in the WDLPS group without the myxoid matrix, and 31 (95 % CI , 15, 47) months in the WDLPS group with the myxoid matrix. The 2-year RFS rates were 75 % and 44 % in the groups without the myxoid matrix and with the myxoid matrix, respectively.Conclusion. The results of the study demonstrated that the presence of the myxoid matrix in WDLPS was associated with poor prognosis. We believe that the presence of the myxoid matrix in WDLPS can serve as an effective morphological marker of a less favorable prognosis for retroperitoneal WLPS .

Correlation of Intravascular Ultrasound with Histology in Pediatric Pulmonary Vein Stenosis

Preliminary intravascular ultrasound (IVUS) images of suspected pediatric intraluminal pulmonary vein stenosis (PVS) demonstrate wall thickening. It is unclear how the IVUS-delineated constituents of wall thickening correlate with the histology. We analyzed six postmortem formalin-fixed heart/lung specimens and four live patients with PVS as well as control pulmonary veins using IVUS and light microscopic examination. In PVS veins, IVUS demonstrated wall thickening with up to two layers of variable echogenicity, often with indistinct borders. Histologically, the veins showed fibroblastic proliferation with areas rich in myxoid matrix as well as areas with abundant collagen and elastic fibers. Discrete vein layers were obscured by scarring and elastic degeneration. A lower reflective periluminal layer by IVUS corresponded with hyperplasia of myofibroblast-like cells in abundant myxoid matrix. The hyper-reflective layer by IVUS extended to the outer edge of the vessel and corresponded to a less myxoid area with more collagen, smooth muscle and elastic fibers. The outer less reflective edge of the IVUS image correlated with a gradual transition into adventitia. Normal veins had a thin wall, correlating with histologically normal cellular and extracellular components, without intimal proliferation. IVUS may provide further understanding of the anatomy and mechanisms of pediatric pulmonary vein obstruction.

Recurrent Myxoid Chondrosarcoma of Mandible: A Case Report

Myxoid chondrosarcoma is a rare malignant neoplasm of bone or soft tissue origin, characterised by the presence of chondroid and myxoid matrix. These are traditionally radio resistant tumours, so wide surgical resection remains the mainstay of treatment. However, radiotherapy is generally advised for high-grade lesions with poor prognostic factors with adjuvant chemotherapy. The treatment decisions are primarily guided by the histological grade, extent of surgical resection, and site of the neoplasm. A case of a 28-year-old male who presented with recurrent painful swelling in the mandibular region is discussed here. To date, very few cases of Extraskeletal Myxoid Chondrosarcoma (EMC) occurring in the mandible region have been reported. So, due to the paucity of available literature, controversies still exist regarding effective treatment approaches in newly diagnosed and recurrent cases. This case reports the clinical presentation, histopathology, radiological characteristics, immune profile, and treatment modalities used in EMC with a comprehensive and relevant review of the literature.

Mesenchymal Chondrosarcoma of the Parotid Gland

Mesenchymal Chondrosarcomas of the parotid are extremely rare tumours. They are broadly classified under extra skeletal form of mesenchymal chondrosarcomas and account for less than 1% of all sarcomas. It is characterized by a multinodular architecture, abundant myxoid matrix, and malignant chondroblast like cells arranged in cords. The tumor is an entity from chondrosarcomas of bones, commonly found in the soft tissues of the lower extremities at 80%. There are very limited reports of this tumor in the head and neck, especially in the parotid gland. We report a rare case of a parotid mesenchymal chondrosarcoma in a 55 years old man and discuss the pathogenesis, clinical presentation and management of such a rare tumour Bangladesh J Otorhinolaryngol; October 2020; 26(2): 158-160

RARE-58. CONGENITAL METASTATIC CHORDOMA OF THE CLIVUS

Chordomas are rare midline axial skeletal neoplasms that typically present in adults. They are infrequent in childhood with typical localization in the spheno-occipital skull base. They are derived from remnants of the embryonic notochord. We present the case of 4 months old girl, who was born with „blueberry muffin” syndrome and was first negatively diagnosed for neuroblastoma and leukemia (two negative skin biopsies were performed) was admitted with axial laxity. In imaging testes there was a tumor of the scull base, metastases in the lungs and kidneys (that were not seen at previous assessments) and a small lesion in the heart. The third biopsy of skin lesion was performed and pathological examination revealed a neoplasm composed of cords, clusters, and chains of multivacuolated cells embedded within a myxoid matrix and separated by fibrous septa. No atypical and dedifferentiated features were present. Mitotic activity was not observed. Neoplastic cells showed the typical cytoplasmic immunostaining for EMA, S100 and cytokeratin AE1/AE3, strong nuclear brachyury expression, and retention of nuclear INI-1 expression. The diagnosis of chordoma was established. Neoplastic tissue and blood samples were obtained for molecular analysis using next generation sequencing, including germline mutations assessment (are ongoing). Chemotherapy as for soft tissue sarcomas was undertaken. Currently a patient is on treatment with improvement of neurological status.

Renal myxoma: An unforeseen diagnosis

Myxomas are rare tumours that can be found in many anatomical locations. There are only 17 cases of renal involvement documented. Our case is an 85 year-old man followed in our consultation with recurrent hematuria after a transurethral resection of a bladder tumour. Evaluation with CT showed a solid lesion with 23 x 18 mm partially obliterating the left inferior calyx. The patient underwent a left nephroureterectomy. Microscopic examination showed a mass within renal parenchyma adjacent to the renal pelvis composed of plump mildly atypical spindle cells distributed in a copious myxoid matrix. Immunohistochemical staining for Vimentine, Pankeratin (AE1/AE3-), CD34, CD31 and smooth muscle actin were negative. With these histopathological and immunohistochemical findings, the case was diagnosed as renal myxoma.

Corded and hyalinized endometrioid carcinoma: a rare case and review of the literature

Background Corded and hyalinized endometrioid carcinoma(CHEC) is a rare morphological variation of endometrioid carcinoma(EC) in the endometrium. Reports of CHEC were very limited. We represent the clinical and pathological findings of this rare endometrioid carcinoma in a 26-year-old woman and reviews the literatures updated on CHEC. Case presentation: A 26-year-old woman presented with abnormal vaginal bleeding for 3 months and the initial cervical biopsy revealed a mullerian mixed tumor in another clinic. Abdominopelvic computed tomography revealed a mass in the uterine cavity and cervix, suggesting a malignant tumor. Histologically, the tumor showed a biphasic pattern characterized by an appearance of 2 components, the conventional endometrioid carcinoma component and sex cord-like component with hyalinization. In the areas of sex cord-like elements, the epithelioid and spindle cells were usually seen around the glands, and mostly arranged in cords or trabeculaes, sometimes embedded within a richly hyalinized collagenous or sometimes myxoid matrix. Tumor cells in the sex cord-like region show a different immunohistochemical expression pattern from conventional adenocarcinoma. Cytokeratin(CK) and vimentin are positive in both components, and vimentin shows more diffuse positivity while CK is more restricted and focally expressed in tumor cells in the sex cord-like region. Complete loss of expression of E-cadherin and epithelial membrane antigen(EMA) was seen in tumor cells in the sex cord-like region whereas it was well preserved in the area of conventional adenocarcinoma. Nuclear expression of β-catenin was noted in tumor cells in the sex cord-like region. P53 was focally positive in both components. Based on histological and immunochemical examinations, the patient was diagnosed with CHEC. Conclusions CHEC is not uncommonly mistaken for a wide variety of diseases. It’s of great significance to raise the awareness of CHEC to avoid over-treatment caused by over-diagnosis, especially in young patients and in curettage. We report one case of CHEC in a 26-year-old woman which was misdiagnosed as a mullerian mixed tumor in the initial curettage specimen. The clinicopathologic, light microscopic, immunohistochemical features of this tumor are described and the differential diagnosis is discussed.

Thyroid Adenoma of Probable Ultimobranchial Body Origin: A Case Report

Solid cell nests are generally believed to represent remnants of the ultimobranchial body, which can be found in the normal thyroid gland, occasionally associated with other branchial pouch remnants such as salivary gland, cartilage, and adipose tissue. We describe the case of a 44-year-old man incidentally found to have a large tumor in the left lobe of the thyroid. The tumor was a circumscribed growth consisting of distinctly lobulated proliferation of solid to cystic epidermoid cell nests and thyroid follicles in a fibromatous stroma, which merged into abundant adipose tissue and focally myxoid matrix. The solid epidermoid cell nests resembled solid cell nests and exhibited a p63+, GATA3+, galectin-3+, TTF1−, PAX8−, thyroglobulin− phenotypes, while the follicles were p63−, GATA3−, galectin-3−, TTF1+, PAX8+, and thyroglobulin+. RAS mutations were not found. This thyroid tumor may represent a hitherto undescribed “ultimobranchial body adenoma” in human.