Solitary Fibrous Tumor of the Peritoneal Cavity and Greater Omentum: Case Report and Review of the Literature.

Background: The solitary fibrous tumor is a rare soft tissue mesenchymal tumor which typically arises from the pleura but may be found anywhere in the body. Abdominal localizations are very rare. The solitary fibrous tumor is classified into two forms, pleural and extrapleural, and morphologically they resemble each other. The diagnostics of the tumor is usually accidental because usually there are no clinical symptoms. The main treatment of the solitary fibrous tumor is the surgical removal of the tumor while radiotherapy treatment and chemotherapy outcomes remain unclear due to the rarity of the tumor and lack of data. Case presentation: We report the case of the 32-year-old man who was diagnosed with the solitary fibrous tumor of the peritoneal cavity. Laparotomy was performed. A grayish-white, stiff, coarse-grained tumor about 11 cm in diameter of the greater omentum was found and radical omentectomy with tumor removal were performed. Postoperative course was uneventful and the patient is well with no signs of recurrence on the CT scan of the chest and abdomen and MRI of the pelvis at 6 months after surgery. Conclusions: The solitary fibrous tumor is a rare condition. It is a borderline-malignant tumor but may cause serious complications if not treated. Due to the absence of clinical symptoms, the tumor is usually detected accidentally. The radical surgical removal of the tumor is the most optimal treatment.

Dental pulp stem cell-derived extracellular matrix: autologous tool boosting bone regeneration.

BACKGROUND AIMS: To facilitate artificial bone construct integration into a patient's body, scaffolds are enriched with different biologically active molecules. Among various scaffold decoration techniques, coating surfaces with cell-derived extracellular matrix (ECM) is a rapidly growing field of research. In this study, for the first time, this technology was applied using primary dental pulp stem cells (DPSCs) and tested for use in artificial bone tissue construction. METHODS: Rat DPSCs were grown on three-dimensional-printed porous polylactic acid scaffolds for 7 days. After the predetermined time, samples were decellularized, and the remaining ECM detailed proteomic analysis was performed. Further, DPSC-secreated ECM impact to mesenchymal stromal cells (MSC) behaviour as well as its role in osteoregeneration induction were analysed. RESULTS: It was identified that DPSC-specific ECM protein network ornamenting surface-enhanced MSC attachment, migration and proliferation and even promoted spontaneous stem cell osteogenesis. This protein network also demonstrated angiogenic properties and did not stimulate MSCs to secrete molecules associated with scaffold rejection. With regard to bone defects, DPSC-derived ECM recruited endogenous stem cells, initiating the bone self-healing process. Thus, the DPSC-secreted ECM network was able to significantly enhance artificial bone construct integration and induce successful tissue regeneration. CONCLUSIONS: DPSC-derived ECM can be a perfect tool for decoration of various biomaterials in the context of bone tissue engineering.

Histologic Lymph Nodes Regression after Preoperative Chemotherapy as Prognostic Factor in Non-metastatic Advanced Gastric Adenocarcinoma.

Background: The study aims to evaluate the lymph node (LN) response to preoperative chemotherapy and its impact on long-term outcomes in advanced gastric cancer (AGC). Methods: Histological specimens retrieved at gastrectomy from patients who received preoperative chemotherapy were evaluated. LN regression was graded by the adapted tumor regression grading system proposed by Becker. Patients were classified as node-negative (lnNEG) in the case of all negative LN without evidence of previous tumor involvement. Patients with LN metastasis were classified as nodal responders (lnR) in case of a regression score 1a-2 was detected in the LN. Nodal non-responders (lnNR) had a regression score of 3 in all of the metastatic nodes. Survival was compared using Kaplan-Meier and Cox regression analysis. Results: Among 87 patients included in the final analysis 29.9 % were lnNEG, 21.8 % were lnR and 48.3 % were lnNR. Kaplan-Meier curves showed a survival benefit for lnR over lnNR (p=0.03), while the survival of lnR and lnNEG patients was similar. Cox regression confirmed nodal response to be associated with decreased odds for death in univariate (HR: 0.33; 95 % CI 0.11-0.96, p=0.04) and multivariable (HR 0.37; 95 CI% 0.14-0.99, p=0.04) analysis. Conclusions: Histologic regression of LN metastasis after preoperative chemotherapy predicts the increased survival of patients with non-metastatic resectable AGC.

Impact of the Interval Between Neoadjuvant Chemotherapy and Gastrectomy on Short- and Long-Term Outcomes for Patients with Advanced Gastric Cancer.

BACKGROUND: The optimal time between neoadjuvant chemotherapy (NAC) and gastrectomy for gastric cancer (GC) remains unknown. This study aimed to investigate the association between the time-to-surgery (TTS) interval and the major pathologic response (mPR). METHODS: In this study, 280 consecutive GC patients who underwent NAC followed by gastrectomy between 2014 and 2018 were retrospectively analyzed by the use of prospectively collected databases from three major GC treatment centers in Lithuania and Estonia. Based on TTS, they were grouped into three interval categories: the early-surgery group (ESG: ≤ 30 days; n = 70), the standard-surgery group (SSG: 31-43 days; n = 138), and the delayed-surgery group (DSG: ≥ 44 days, n = 72). The primary outcome of the study was the mPR rate. The secondary end points were postoperative morbidity, mortality, oncologic safety (measured as the number of resected lymph nodes and radicality), and long-term outcomes. RESULTS: The mPR rate for the ESG group (32.9%) was significantly higher than for the SSG group (20.3%) or the DSG group (16.7%) (p = 0.047). Furthermore, after adjustment for patient, tumor, and treatment characteristics, the odds for achievement of mPR were twofold higher for the patients undergoing early surgery (odds ratio [OR] 2.09; 95% conflidence interval [CI] 1.01-4.34; p = 0.047). Overall morbidity, severe complications, 30-day mortality, R0 resection, and retrieval of at least 15 lymph nodes rates were similar across the study groups. In addition, the long-term outcomes did not differ between the study groups. CONCLUSIONS: This study suggests that an interval of more than 30 days between the end of NAC and gastrectomy is associated with a higher mPR rate, the same oncologic safety of surgery, and similar morbidity and mortality.

Maxillary epithelioid hemangioendothelioma: an especially rare malignant tumor mimicking periodontal disease.

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is an especially rare, low-grade malignant vascular tumor that, according to WHO classification, is described as locally aggressive tumor with possible metastasis and makes up 1% of all vascular tumors. EHE is characterized by the accumulation of round, eosinophil-infiltrated endothelium cells; with vacuolation of their cytoplasm; frequent angiocentric inflammation; and myxohyaline stroma. This tumor is usually found in the liver, lungs, and bones and is especially rare in the mouth. CASE PRESENTATION: We present an 18-year-old Caucasian female whose oral cavity lesion had been misdiagnosed as marginal periodontitis. The patient was treated improperly for 2 years until she was referred to a maxillofacial surgeon. The patient complained only about gingival recession in the palatal area of her upper-right-side 13th, 14th, and 15th teeth. The lesion's clinical appearance was of locally ulcerated painless lesion that affect the underlying bone as seen in X-rays in the palatal side of the right canine and the first and second premolars. Patient underwent surgery for her present defect and reconstruction using allogenic bone transplant. The diagnosis of EHE was based on the bony destruction as seen in x-rays and in the accumulation of tumor cells that were 100% positive to CD31; CD34 and ERG to endothelial markers. During the 31-month follow-up period, the patient exhibited no clinical and radiographic complications. CONCLUSIONS: With this clinical case, we demonstrate that this rare tumor must be included in differential diagnoses of periodontal pathologies to perform histomorphological examination in a timely manner, which could lead to correct diagnosis and adequate treatment.

Primary Ewing's sarcoma in a small intestine - a case report and review of the literature.

BACKGROUND: Ewing's sarcoma usually presents in paediatric patients with its primary location being bone tissue. Nevertheless, we present such an adult case which arises from the small intestine. We registered thirty one cases of such origin published so far excluding ours. CASE PRESENTATION: We report a case of 30 year old female who was admitted due to the persistent anaemia. Whole body computed tomography scan revealed abdominal mass in her left upper abdominal compartment. Surgery on the mass originating from jejunum was performed, although due to extremely complicated postoperative period and rapid dissemination no additional therapy had been performed. The tumour was positive for CD99, ERG, CD56, Synaptophysin, PanCK, Cam5.2. CONCLUSION: Extraosseus Ewing's sarcoma is extremely rare entity, with poor prognosis.

Significance of BRAF V600E Mutation and Cytomorphological Features for the Optimization of Papillary Thyroid Cancer Diagnostics in Cytologically Indeterminate Thyroid Nodules.

BACKGROUND: Ultrasound guided fine needle aspiration biopsy with cytologic analysis is an initial step in diagnostic of thyroid nodules. Unfortunately, up to 30% of biopsies are indeterminate and diagnostic surgery is required. The aim of this study was to estimate the diagnostic value of BRAF V600E mutation status combined with cytomorphological features for diagnosis of papillary thyroid cancer (PTC) in cytologically indeterminate thyroid nodules. METHODS: A prospective study analyzed patients who had ultrasound suspicious thyroid nodules, underwent fine needle aspiration and cytological examination, and were classified according to the Bethesda system. Patients from indeterminate diagnostic categories were examined for BRAF V600E mutation and 22 cytomorphological features, and underwent thyroid surgery. A binary logistic regression model was used to evaluate the diagnostic utility. RESULTS: A total of 219 patients met study criteria. After histological examination, 77 (35.2%) patients were diagnosed with PTC and 142 (64.8%) with benign nodular thyroid disease. According to logistic regression model, significant features for PTC diagnosis were: liquid colloid consistency, papillary structures, eosinophilic colloid bodies, and BRAF V600E mutation. Risk groups classified by this model have sensitivity of 80.5% (95% CI: 69.9 to 88.7), specificity of 99.3% (95% CI: 96.1 to 100), positive predictive value of 98.4% (95% CI: 89.8 to 99.8), negative predictive value of 90.4% (95% CI: 85.7 to 93.7), and accuracy of 92.7% (95% CI: 88.4 to 95.8) for PTC diagnosis. CONCLUSIONS: Evaluation of BRAF V600E mutation status combined with cytomorphological features for diagnosis of PTC in cytologically indeterminate thyroid nodules can significantly improve diagnostic accuracy and reduce the number of diagnostic operations (calculator available at www.ptc-calc.we2host.lt).

Oral lichen planus: a 4-year clinical follow-up study.

BACKGROUND/AIM: This study aimed to examine oral lichen planus (OLP) cases histopathologically and cytologically, describe different clinical aspects of OLP manifestations, and associate different OLP clinical forms with self-reported oral health and with local and systemic risk factors. MATERIALS AND METHODS: All patients with a referral diagnosis of OLP received biopsies. The histological evaluation assessed infiltration with lymphocytes, epithelial hyperplasia, and hyperkeratosis. Histology was used to validate the OLP diagnosis and to evaluate the malignant transformation of OLP lesions. Clinical manifestations of OLP and potential local oral environment-related risks were clinically assessed. Systemic risks were evaluated by a structured questionnaire. RESULTS: A total of 133 patients with a histologically confirmed OLP diagnosis were followed. There were more females (N = 109) than males (N = 24) and the majority of patients were older than 50 years. The reticular form (45.1%) was the most prevalent clinical form followed by the erosive-ulcerative form (33.8%). The histological characteristics were most pronounced in the erosive-ulcerative form. Of the OLP cases, 2.3% had a malignant transformation. CONCLUSION: Reticular and erosive-ulcerative were the most prevalent OLP forms. There were more female than male OLP patients. Except for medication use, there were no statistically significant differences among different clinical OLP forms in either local or systemic risk factor distributions.

Small Intestinal Inflammatory Myofibroblastic Metastasis in the Left Ventricle.

Inflammatory myofibroblastic tumors (IMTs) are rare and usually appear in childhood and adolescence. These tumors often take a benign course, but tend to reoccur in a quarter of cases and metastasize locally. Distant IMT metastases are extremely uncommon. To our knowledge, metastatic spread to the heart has not been reported. We present a case of a 43-year-old woman with small intestinal IMT metastatic spread to the cavity of the left ventricle, stomach, liver, vertebra, and pelvic bones. The cardiac tumor was resected, and the patient survived for 9 months. She dies because the progression of IMT at other sites.

Tumour-induced osteomalacia: a literature review and a case report.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterised by severe hypophosphataemia and osteomalacia, with renal phosphate wasting that occurs in association with tumour. The epidemiology likewise aetiology is not known. The clinical presentation of TIO includes bone fractures, bone and muscular pains, and sometimes height and weight loss. TIO may be associated with mesenchymal tumours which may be benign or malignant in rare cases. Mesenchymal tumour itself may be related to fibroblast growth factor 23 (FGF23), which is responsible for hypophosphataemia and phosphaturia occurring in this paraneoplastic syndrome. Hypophosphataemia, phosphaturia and elevated alkaline phosphatase are the main laboratory readings that may lead to more precise investigations and better diagnosis. Finding the tumour can be a major diagnostic challenge and may involve total body magnetic resonance imaging, computed tomography and scintigraphy using radiolabelled somatostatin analogue. The treatment of choice for TIO is resection of a tumour with a wide margin to insure complete tumour removal, as recurrences of these tumours have been reported. We provide here an overview on the current available TIO case reports and review the best practices that may lead to earlier recognition of TIO and the subsequent treatment thereof, even though biochemical background and the long-term prognosis of the disease are not well understood. This review also includes a 4-year-long history of a patient that featured muscular pains, weakness and multiple stress fractures localised in the hips and vertebra with subsequent recovery after tumour resection. Because the occurrence of such a condition is rare, it may take years to correctly diagnose the disease, as is reported in this case report.

Fatal pulmonary complication during induction therapy in a patient with ANCA-associated vasculitis.

ANCA-associated vasculitis (AAV) is an inflammatory systemic disorder affecting small to medium sized vessels and likely leading to any organ dysfunction. Adequate treatment is important to avoid mortality or severe organ damage. In most cases initial treatment (induction therapy) allows to achieve remission. Induction therapy leads to immunosuppression and may cause severe infections. However, in vasculitis patients even an intensive immunosuppressive therapy is rarely complicated by an invasive fungal infection. We present a case in a 29-year old male patient with newly diagnosed AAV. He suffered a fatal pulmonary complication of the induction immunosuppressive treatment. Pathological (infectious) changes in the lungs were misinterpreted as progression of the vasculitis and he died due to disseminated angioinvasive aspergillosis. A clinical course, imaging and histopathology of this case are described and discussed.

Asymptomatic heterotopic pancreas in Meckel's diverticulum: a case report and review of the literature.

INTRODUCTION: Heterotopic pancreas is defined as pancreatic tissue without a real anatomical or vascular connection to the pancreas. It can be found in the stomach, duodenum, jejunum, ileum, Meckel's diverticulum, colon gall bladder, umbilicus, fallopian tube, mediastinum, spleen and liver. Complications of heterotopic pancreas are inflammation, bleeding, obstruction, malignant transformation, carcinoid syndrome, jejunojejunal intussusception and ileus, but it is usually asymptomatic and diagnosed only during examinations for other diseases. CASE PRESENTATION: An 81-year-old Lithuanian woman was diagnosed with caecal cancer and had undergone elective surgery. A right hemicolectomy was performed and a Meckel's diverticulum was observed and excised. Histological results showed a poorly differentiated G3 adenocarcinoma of her large intestine and heterotopic pancreas tissue in the Meckel's diverticulum and mesenteric adipose tissue. CONCLUSIONS: Asymptomatic heterotopic pancreas is rarely diagnosed, and usually found incidentally during surgical or diagnostic interventions. Although it has no symptoms, heterotopic pancreas found during surgical procedures should be excised.

Echinococcus multilocularis infection of the liver presenting as abdominal wall fistula.

Echinococcus multilocularis causes infection where the most commonly affected organ is the liver, followed by the lung, kidney, bone and the brain. Other sites such as the heart, spleen, pancreas and soft tissues are very rarely affected. Surgical treatment combined with chemotherapy using various technical approaches remains the main therapeutic modality for echinococcal liver disease. To the best of our knowledge there are less than five clinical cases of cutaneous presentation of liver alveolar echinococcosis described. We present a unique case of liver echinococcosis presenting as recurrent abdominal wall fistula and abscess in a 29-year-old man. Diagnosis was based on CT imaging, serological analysis and histological findings from the fistula. Medical treatment with albendazole was initiated and liver resection was performed. The patient has no symptoms and signs of recurrence 1 year after operation, while still on albendazole therapy. This case description highlights the importance of early suspicion and treatment of unusual echinococcosis clinical presentations.

Solid Pseudopapillary Neoplasm of the Pancreas: Analysis of Seven Cases.

BACKGROUND: The purpose of this study was to describe as well as compare our surgical treatment experiences of solid pseudopapillary neoplasms (SPN) of the pancreas and to provide a review of the literature. METHODS: A retrospective analysis of data from Vilnius University Hospital Santariskiu Klinikos (VUH SK) and of the literature, which was researched using Karger Publishers, Springer Science, BioMed Central, and disserCat databases, was conducted. RESULTS: From 2001 to 2012, seven cases were identified with pathologically confirmed SPN diagnosis. A precise preoperative diagnosis was made by computertomography and magnetic resonance imaging. The median diameter of the tumors was 6.36 cm (range 1.5-12 cm). Surgical treatment was undertaken for all patients. Results of the immunohistochemical analysis confirmed a nuclear accumulation of ß-catenin. The Ki-67 level was 1-2% in all of the cases. According to our collected data, all types of histological analysis revealed decent prognostic behavior with low mitotic activity (1-2 mitoses per 50 high power fields). Besides, angioinvasion, perineural invasion, and outside capsule invasion were not detected. CONCLUSIONS: There was no correlation between more aggressive types of SPN and tumor size, localization, age, and gender.

Clear cell sarcoma expressing a novel chimerical transcript EWSR1 exon 7/ATF1 exon 6.

Clear cell sarcoma harbours recurrent translocation, resulting in EWSR1/ATF1 or less commonly EWSR1/CREB1 fusion. To date, six types of EWSR1/ATF1 fusion have been reported, of which three are in-frame and encode functional proteins. We present a reverse transcription - polymerase chain reaction analysis of a tumour near the hallux of the right foot. The sequencing of obtained fragments revealed the presence of a novel chimerical transcript-the in-frame fusion between EWSR1 exon 7 and ATF1 exon 6 that represents the fourth in-frame type of EWSR1/ATF1 fusion identified in clear cell sarcomas.

Fatal case of ectopic enterobiasis: Enterobius vermicularis in the kidneys.

Enterobius vermicularis is one of the most common intestinal parasites found in humans. They commonly infest the terminal ileum and large intestine, and are usually considered an innocuous parasite that can be easily eradicated with proper treatment. However, extraintestinal migration of worms, although very rare, may lead to severe health disorders or even death. This article, reports the first fatal case of ectopic enterobiasis known to the authors, which developed in an adult patient with E. vermicularis infection, causing perforation of the large intestine and generalized bacterial peritonitis. Despite emergency laparotomy, the patient died from septic shock on the day after surgery. During pathological examination, worms were found not only in the large intestine, but also in the renal parenchyma; worm eggs were found deposited in the lungs as well.

Discovery of two novel EWSR1/ATF1 transcripts in four chimerical transcripts-expressing clear cell sarcoma and their quantitative evaluation.

The most common recurrent translocation in clear cell sarcoma t(12;22)(q13;q12) results in an EWSR1/ATF1 chimeric gene. We present a molecular analysis of tumor overgrowing right proximal tibia with bone destruction metastatic to two groin lymph nodes. Fluorescent in situ hybridization analysis performed on paraffin-embedded tissue sections of primary tumor sample indicated one rearranged locus of EWSR1 gene and one additional red signal. Reverse transcription-polymerase chain reaction analysis revealed the presence of four different EWSR1/ATF1 chimerical transcripts in the tumor sample as well as in both metastatic lymph nodes. Two previously described transcripts EWSR1exon7/ATF1exon5 and EWSR1exon8/ATF1exon4, and two novel transcripts EWSR1exon7/ATF1exon4 and EWSR1exon9/ATF1exon4 were identified. Both novel transcripts were out-of-frame fusions and, therefore, most likely had limited biological impact in oncogenesis of clear cell sarcoma. Quantitative evaluation demonstrated unequal distribution of these transcripts, with EWSR1exon8/ATF1exon4 type being overexpressed.

Renal amyloidosis in a child with chronic granulomatous disease and invasive aspergillosis.

A boy who had been diagnosed with chronic granulomatous disease (CGD) at the age of 6.5 years had a medical history of multiple bacterial infections, including pneumonia, staphylococcal liver abscesses and septicemia, from birth. At the age of 10 years and 4 months he developed an infection that was accompanied by high fever and pulmonary, mediastinal and paravertebral infiltrations. Aspergillus niger was cultured on bronchial secretions obtained by bronchoscopy. Shortly thereafter, proteinuria manifested and progressed to the nephrotic level. A skin biopsy indicated a diagnosis of amyloidosis. An anti-fungal treatment with amphotericin B and other agents, along with surgical pus drainage, intravenous leukocyte mass, interferon-gamma and immunoglobulin infusions, was ineffective, and the patient eventually died from multi-organ failure. The postmortem examination revealed the presence of disseminated aspergillosis and systemic amyloidosis. Although no direct evidence is available that would confirm the causative role of aspergillosis in the development of systemic amyloidosis, to the best of our knowledge this is the first report of a CGD case with complications of both invasive aspergillosis and systemic amyloidosis.

[Treatment of rectal tumors with transanal endoscopic microsurgery]. / Tiesiosios zarnos naviku gydymas transanaline endoskopine mikrochirurgija.

OBJECTIVE: The aim of this study was to assess one and a half years experience gained in Lithuania while treating rectal tumors with transanal endoscopic microsurgery in the Centre of Abdominal Surgery of Vilnius University Hospital "Santariskiu klinikos". MATERIALS AND METHODS: The patients who had rectal adenomas and low-risk T1 carcinomas of good or moderate differentiation, with no lymphatic and vascular invasion were selected for surgery. Tumor stage was determined by transanal endosonoscopy and rectoscopy with multiple macrobiopsies before surgery. RESULTS: A total of 47 patients were operated on. The average tumor size was 3.4+/-1.4 cm (ranged from 1 to 7 cm). Overall 25 (52.1%) carcinomas and 23 (47.9%) adenomas were removed. Pre-operative diagnoses did not correspond to the final clinical diagnoses in 14 (29.8%) cases. Forty-three (89.6%) radical operations (R0) and 5 (10.6%) doubtful complete operations (RX) were performed. One (2.1%) intra-operative complication and one (2.1%) post-operative complication were observed. After the removal of Ca T2 three patients underwent adjuvant radiotherapy. Twenty-six patients were followed up for 3-17 months after operation: 17 after removal of cancer and 9 after removal of adenoma. One (2.1%) recurrence of a tubulovillous adenoma was diagnosed. No other complications were reported. CONCLUSIONS: Initial results of transanal endoscopic microsurgery obtained while treating rectal adenomas and low-risk T1 cancers are promising. The low rate of complications and recurrences in this group offers many hopes. The experience of the treatment of T2 cancers with transanal endoscopic microsurgery and adjuvant radiotherapy is limited but the results are encouraging. It is obvious that the results of randomized and controlled trials need to be awaited before definite conclusions can be drawn.