Approach to Primary Vertebral Tumors in the Light of the 2020 Updated World Health Organization Classification of Bone Tumors.

AIM: To define a new approach for primary vertebral tumors by discussing them in the light of 2020 World Health Organization (WHO) classification of bone tumors. MATERIAL AND METHODS: In this study, we have discussed primary vertebral tumors in light of the 2020 Updated WHO Classification of Soft Tissue and Bone Tumors. RESULTS: Chondroblastoma and chondromyxoid fibroma has been classified in the benign category, while synovial chondromatosis has been moved from the benign category to the intermediate category. In the updated classification, grade I chondrosarcoma has been classified in the malignant category with grade II?III chondrosarcomas. Minor amendments have been made for osteosarcoma subtypes. Neoplasms of undetermined origin, such as aneurysmal cysts, simple bone cysts, fibrosis dysplasia, and osteofibrous dysplasia, have been classified as neoplastic lesions. Chordomas have been classified into ?not otherwise stated,? poorly differentiated chordomas, and dedifferentiated chordomas. Ewing?s sarcomas have been classified in a separate section for undifferentiated, small, round cell sarcomas of the bone and soft tissue. In this section, three distinct subsets different from Ewing?s sarcoma have been discussed. CIC-rearranged sarcoma, BCOR-rearranged sarcoma, and round cell sarcomas with EWSR1 gene fusion with non-ETS family members. CONCLUSION: In this study, we have reviewed the new classifications and discussed their effect on decision making in spinal oncologic surgery.

Human chorionic gonadotrophin secreting adrenocortical neoplasm presenting with peripheral precocious puberty in an infant.

Adrenocortical tumor (ACT) is a rare malignant tumor which usually present with Cushing syndrome and virilization. Paraneoplastic syndromes (PNS) due to neoplasms can occur with peptides or cytokines secreted by the tumor. Here, we report a 13-month-old-male presented with severe masculinization. He had signs of precocious puberty with enlarged testicles, very high testosterone levels but low levels of gonadotrophins, and elevated ß-hCG. He underwent a left nephrectomy. The histopathological evaluation revealed a diagnosis of adrenocortical neoplasm. The levels of androgens and ß-hCG normalized after the resection of tumor, and the clinical findings improved within few months. We report the first pediatric patient with peripheral precocious puberty due to an ACT that secretes ß-hCG as PNS. A ß-hCG secreting ACT can cause severe virilization due to increased gonadal androgens stimulated by ß-hCG as well as due to increased adrenal androgens from the tumor.

Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion.

Biphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, first described in 2012. It involves both myogenic and neural differentiation and is characterized by PAX3 rearrangement. MAML3 is the most frequent fusion partner of PAX3; however, its partner remains unidentified in a subset of cases. These tumours have significant local recurrence rates but lack metastatic potential. Here, we report a case of BSNS with PAX3/FOXO1 fusion and discuss its clinicopathological features and differential diagnosis.

Intra-Articular Tenosynovial Giant Cell Tumor Mimicking Septic Arthritis: A Report of Two Cases.

Introduction: Intra-articular tenosynovial giant cell tumor (IATGCT) is a type of tenosynovial giant cell tumor that typically occurs in the synovial tissues of large joints. It is also known as pigmented villonodular synovitis. Acute onset of the pain with irritable hip symptoms is very rare. In this paper, we presented two adolescents with acute onset of hip pain mimicking septic arthritis diagnosed with intra-articular tenosynovial giant cell tumor. Case Report: Healthy two adolescents, one male (14-year-old) and the other girl (15-year-old) with no history of the previous trauma or significant comorbidities were presented complaining of acute onset of hip pain to our emergency room. Although initial possible diagnosis was septic arthritis for both cases, laboratory findings were unequivocal for septic arthritis and magnetic resonance imaging (MRI) showed an intra-articular nodular mass. An open resection was performed and pathological evaluation revealed the masses to be intra-articular tenosynovial giant cell tumor. After 26 and 17 months follow-up there was no pain neither with activity nor in rest, hip range of motion was within normal ranges. There was no recurrence, avascular necrosis or destruction detected on control MRI for both patients. Conclusion: IATGCT is a rare disease of the pediatric population involving the hip. Inflammation or infarction of the lesion can trigger irritable hip findings in children. This diagnosis should be kept in mind mainly in cases with serohemorrhagic aspirate and unequivocal laboratory findings.

A novel therapeutic approach to NASH: Both polyethylene glycol 3350 and lactulose reduce hepatic inflammation in C57BL/6J mice.

BACKGROUND: The gut-liver axis is one of the most emphasized topics in the pathogenesis of non-alcoholic fatty liver disease (NAFLD). Intestinal microbiota dysbiosis has been shown to be a predictor of disease severity and progression to fatty liver disease. Therefore, research addressing gut-based therapies has become popular. OBJECTIVES: To investigate the effect of lactulose and polyethylene glycol 3350 (PEG 3350) in mice with induced obesity and NAFLD at a non-diarrheal dose. MATERIAL AND METHODS: Thirty-six C57BL/6J male mice were divided into 6 groups. The first 2 groups (n = 6 each) were used as an induced obesity model (group A) and NAFLD model (group B) for 8 weeks. The remaining 24 animals were categorized into control diet group, high-fat diet (HFD) group, HFD + lactulose group, and HFD + PEG 3350 group. Serum and liver tissue samples were obtained for biochemical and histopathological analyses, respectively. RESULTS: The HFD + lactulose treatment group displayed a significant decrease in liver weight (1.3 (1.3-1.4) kg compared to 1.8 (1.6-1.9) kg) and NAFLD activity score (NAS) (1.5 (1.0-3.0) compared to 5.0 (4.0-5.0), respectively; p = 0.0043, p = 0.0021) when compared with the HFD group. However, a decrease in body weight (35.0 (34.6-36.0) kg compared to 40.9 (34.7-41.9) kg) and hepatosteatosis (HS) rate (33.3% compared to 100.0%) were not statistically significant (p = 0.1796, p = 0.0606, respectively). The HFD + PEG 3350 treatment group showed a statistically significant decrease in body weight (32.4 (30.2-33.9) kg compared to 40.9 (34.7-41.9) kg), liver weight (1.5 (1.3-1.5) kg compared to 1.8 (1.6-1.9) kg), HS rate (16.7% compared to 100.0%) and NAS (0.5 (0.0-1.0) compared to 5.0 (4.0-5.0); p = 0.0086, p = 0.0086, p = 0.0151, and p = 0.0021, respectively) when compared with the HFD group. CONCLUSIONS: We demonstrated that non-diarrheal dose of lactulose and PEG 3350 reduced hepatic inflammation in mice with induced NAFLD. It was also observed that PEG 3350 decreased HS and body weight. We believe these mechanisms can be utilized as novel therapeutic approaches in NAFLD in prospective human studies.

Ossified uncertain malignant potential gastric glomus tumor with tumor thrombus.

Glomus tumor is a rare mesenchymal tumor composed of perivascular glomus bodies. The most common presentation area of these tumors is peripheral soft tissue, particularly in the distal part of extremities. They rarely can occur in the gastrointestinal tract and the most common location is the stomach. Preoperative diagnosis of this tumor can be difficult because of rarity and overlapping features with other mesenchymal lesions with regard to clinical and pathological findings. Therefore, to exclude differential diagnosis and make a definitive diagnosis is possible only with histopathological examination. In this case, we evaluated glomus tumor of stomach according to 2019 WHO Digestive System Tumors and accurate diagnosed was Uncertain Malignant Potential Gastric Glomus Tumor.

Superficial CD34 positive fibroblastic tumor: report of three cases and review of the literature.

Superficial CD34 positive fibroblastic tumor (SCPFT) is a recently recognized, unique neoplasm with distinctive histomorphological features such as high pleomorphism, low mitotic rate, and diffuse CD34 reactivity. Hereby we present three cases of our experience with clinicopathological, morphological, and immunohistochemical characteristics. The patients were a 31-year-old female, 53-year-old female, and 33-year-old male. The tumors were all superficially located; left forearm, medial aspect of the left ankle, and left thigh, respectively. Histomorphologically they had expansile and focal infiltrative growth pattern consisting of highly pleomorphic spindle cells with intranuclear inclusions, yet low mitotic rate. Tumoral cells showed strong and diffuse reactivity for CD34. One of our cases showed focal and weak reactivity for pancytokeratin. Unlike the other two tumors, one case was positive for desmin. During the clinical follow-up, one case showed local recurrence four times. SCPFT is a newly recognized, borderline mesenchymal neoplasm of soft tissues that can show local recurrence or even rarely metastasize. To the best of our knowledge, this three case series is the first to be reported from Turkey. Our aim to report these three cases was to make contribution to the literature about this rare entity and increase awareness.

Dural prostate metastasis presenting as a subdural hematoma.

The incidence of subdural hematoma is approximately 13.1/100.000 per year. Subdural hematoma due to skull and dura mater metastases is rare. In this study, a 71-year-old patient with prostate adenocarcinoma who presented with chronic subdural hematoma due to skull bone and dura mater metastasis is presented.

Solid pseudopapillary tumor of the pancreas: a case series of 9 patients.

BACKGROUND/AIMS: Solid pseudopapillary tumor is a rare exocrine tumor of the pancreas. There is no clear consensus on its etiology, origin and treatment. In this study, the clinical, pathological and immunohistochemical features of nine patients with solid pseudopapillary tumor were re-evaluated in view of the current literature findings. MATERIALS AND METHODS: We studied nine cases diagnosed with solid pseudopapillary tumor between 2005 and 2010. The clinical, pathological and laboratory data were analyzed. RESULTS: On microscopy, all tumors had well-defined borders and were separated from surrounding pancreatic tissue by a thick fibrous capsule. The tumor consisted mainly of pseudopapillary structures with focal solid areas accompanied by wide hemorrhagic and cystic regions. The typical morphological features were present to varying degrees. Of the nine cases, one relapsed approximately two years after the diagnosis, and our laboratory also evaluated the surgical specimen of local recurrence. CONCLUSIONS: While some new light has been shed on the clinicopathological features of solid pseudopapillary tumor concerning its etiology, origin and treatment methods, there is much to be understood. Further studies focusing on genetics, pathogenesis and prognosis are needed for a better understanding of this entity.

Clinical and pathological features of 31 cases of lipedematous scalp and lipedematous alopecia.

Little is known about lipedematous scalp (LS) and lipedematous alopecia (LA). We investigated the clinical and histopathological features of LS and LA with a 7-year retrospective re-evaluation of 31 patients. 23 cases were LS and 8 LA, with 25 females and 6 males. The overweight and obese groups contained 15 patients with 16 within the normal weight range. Scalp thickness varied between 9-18 mm in our patients by magnetic resonance imaging. Thickening of the subcutaneous adipose tissue layer was present in all cases. Dermal edema was seen in 22 patients, lymphatic dilatation in 17 and elastic fiber fragmentation in 21. When the relationship between dermal edema and elastic fibers was investigated, elastic fiber fragmentation was found in 86.4% of cases with dermal edema. Collagen fragmentation and coarsening were seen in two cases, and collagen was normal in 24 cases. The number of follicles was decreased in 9 cases and normal in 17. The clinical and histopathological findings were not statistically different between LS and LA groups (p>0.05). The majority of the patients in our study were females, suggesting an underlying hormonal pathology. The association with obesity suggested that anatomical differences can be present in lipid distribution. Dermal edema and lymphatic dilatation suggested the primary pathology is lymphatic system.

Schwannoma of the tongue in a child.

Schwannomas are tumors of the peripheral nerves originating in the nerve sheaths that account for 1% of benign tumors located in the oral cavity. The tongue as a whole is the most common location for intraoral schwannomas to occur; however, it is quite rare to diagnose schwannoma in children. We are contributing a report of a 13-year-old child with a 1-year history of slowly growing swelling on the anterior part of corpus of the tongue. The patient complained of the disturbance to mastication and phonation. Diagnosis was confirmed by excisional biopsy. Histologic identification of Antoni A and B areas along with strong and diffuse staining with S-100 stain pathologically completed the diagnosis of schwannoma.