Inflammatory Pseudotumor/Inflammatory Myofibroblastic Tumor of Spleen – A Case Report

BACKGROUND: Splenic inflammatory pseudotumor (IPT)/inflammatory myofibroblastic tumor (IMT) is a rare pseudotumor/tumor of unknown origin, which is usually benign, although atypical and aggressive cases have been reported. It is a lesion composed of proliferated myofibroblastic cells (hence IMT by some authors) with admixed pleomorphic inflammatory cells of varying proportions. CASE REPORT: Herein, we report a case of 61-year-old male patient with ill-defined abdominal discomfort and no other symptoms and signs. Clinical and imaging investigations revealed a mass in the spleen that was equivocally interpreted as secondary neoplasm, although primary neoplasm of the spleen was not excluded by the radiologists. Splenectomy was performed and on gross examination a well demarcated greyish-livid tumor measuring 3.5 cm × 3 cm × 3 cm was discovered. Microscopic examination showed proliferation of loosely arranged spindle cells admixed with inflammatory cells (histiocytes, lymphocytes, neutrophils, eosinophils, occasional plasma cells, and/or plasmacytoid cells) with varying density and multifocal clustering, multifocal hemorrhage, and fibrinoid-like deposition. We performed additional histochemical and immunohistochemical stainings which were consistent with the diagnosis of IPT/IMT. Next-generation sequencing (TruSight Tumor 15) showed common TP53 polymorphism (c.215C>G; p.Pro72Arg) along with several intronic and synonymous single nucleotide variations (SNVs), as well as five low confidence missense SNVs. Sixteen months after the operation the patient has uneventful follow-up. CONCLUSION: Although the incidence of IPT/IMT is low, awareness of its existence is necessary. The prognosis is favorable following splenectomy in most cases. Careful microscopic examination of the specimen is mandatory, due to possible misdiagnosis. We believe that extensive NGS analysis on archive samples would provide more data about the spectrum of possible genetic changes in lesions like IPM/IMT

Survival of Advanced Stage High-Grade Serous Ovarian Cancer Patients in the Republic of Macedonia

AIM: The primary objective of the study was to evaluate the overall survival of women with advanced stage (Stage IIIA-IV) high-grade serous ovarian cancer in MacedoniaMATERIALS AND METHODS: The study was a cross-sectional medical record review of patients diagnosed with advanced stage HGSC. Patients were deemed eligible for inclusion if they were diagnosed with an advanced stage (Stage IIIA-IV) HGSC of the ovary, fallopian tube or peritoneum between 2009 and 2015.  The data were analyzed in a descriptive fashion and summary statistics were provided, as appropriate. Survival was calculated using the Kaplan-Meier method.RESULTS: A total of 81 eligible patients were identified and included in the study. The average overall survival in the studied cohort was 46.59 months (95%CI = 39.11-54.06). Patients that were optimally debulked and patients that had a platinum-free interval larger than 12 months had significantly longer survival in the current series (p < 0.001).CONCLUSION: the average overall survival of advanced stage HGSC patients in the studied series was 46.59 months (95%CI = 39.11-54.06). Patients aged 65 years or younger tended to live approximately ten months longer than patients older than 65 years, but this difference was not statistically significant. There was no difference in HGSC survival in the groups of patients with grade 2 and grade 3 disease. However, optimal surgical debulking and platinum sensitivity were associated with significantly better overall survival

A Rare Case of Soft Tissue Erdheim Chester Disease: Diagnostic Dilemma and Management

BACKGROUND: Erdheim Chester disease (ECD) is a rare form of non-Langerhans histiocytosis that still presents a diagnostic and clinical dilemma. CASE PRESENTATION: We present a rare case of ECD, young 31 male with atypical localisation and soft tissue presentation and no bone involvement. He started clinical investigations due to subcutaneous tumour mass in the lumbar spine that caused severe back pain. Skin biopsy revealed ECD with Immunohistochemistry CD68+, CD10+, CD11c+, vimentin+, S100A4+. Activating BRAFV600E mutation was positive from the tumour tissue. The patient was referred to the haematology department. PET CT was performed for initial disease staging. Treatment was started with corticosteroids (methylprednisolone 0.5 mg/kg per day), and after 7 days, a significant clinical improvement was noticed in terms of pain disappearance with no need for pain killers. After two weeks, treatment with interferon Alfa (IFN-α) was started in a dose of 3 million units 3 times per week. After 4 months of interim treatment PET, CT revealed a significant reduction of the tumour mass. Therapy with IFN-α was continued, and the patient is still clinically in good condition. CONCLUSION: It can be concluded that shortening the time of diagnosis of ECD is essential in treatment outcome of this disease. Still, large studies have to confirm the best treatment of this rare condition

The outcome of Pregnancy with Fetal Primitive Neuroectodermal Tumor

BACKGROUND: Fetal intracranial tumours are very rare. The overall incidence is 0.34 per one thousand live birth newborns. According to the new classification of central nervous system tumour (2016), a primitive neuroectodermal tumour of (PNETs) is an embryonal tumour group; these are tumours with high malignancy and belong to group IV (WHO). In our case, we will present a case of PNETs in 28 gestation week old fetus, diagnosed antenatally and confirmed postnatally.CASE REPORT: We present the third pregnancy in 29 years old patient, with two previous term deliveries of healthy newborn. She came to University clinic at 27+3 gestational week for fetal hydrocephalus. After an ultrasound and MRI scan, possibilities were explained to the parents. During the medico-ethical counselling, explain to the parents the need for operation and the possibility of postoperative adjuvant therapy, quality of life with potential future disabilities. They choose to terminate the pregnancy. Postmortem the diagnosis was PNETs. Summary of analysis: peripheral neuroectodermal tumour with ganglion and neuronal differentiationCONCLUSION: Antenatal management depends on the gestational week in the time of diagnosis and the decision of parents. If the lesion is before viability fetus, it should be offered termination of pregnancy. Another important factor is the mode of delivery, because of increased intracranial pressure although this aggressive combined modality of treatment, recurrence is often. Tree year of survival is between 53% and 73% when the adjuvant radiotherapy is included. For that, they should be diagnosed as soon as possible before achieving fetal viability. Only 18% of those tumours presenting in the first year of life are diagnosed before or at delivery

Adenomatoid tumor of the adrenal gland in young woman: from clinical and radiological to pathological study

Adenomatoid tumors are neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Extragenital localization sites such as adrenal glands are rare but have been reported. When found in the adrenals, they represent great clinical, radiological and pathological diagnostic challenge, with wide range of differential diagnoses to be considered. We present a case of a 30 years old female, with incidental ultrasound finding of unilateral tumor in the right adrenal gland. Multi slices CT scan was of value in localizing this tumor, but not in the precise diagnosis. The tumor ranged from 5.6 cm to 6.4 cm in greatest diameter. Clinical and hormonal examinations excluded Sy. Cushing, M. Conn and pheochromocytoma. The patient underwent laparoscopic right adrenalectomy. A large tumor (d: 8×7×3 cm) was removed showing no infiltration of the adrenal cortex or medulla, or extra-adrenal extension into the periadrenal adipose tissue. Histological examination showed numerous cystic spaces lined by flattened cubical epithelial cells. The small cystic spaces were separated by edematous fibrovascular stroma with rare epithelial cells with vacuolated cytoplasm. Immunohistochemical staining was positive with vimentin (+), S100 (+), MCA mesothelial Ag (+), CD 68 (+) and negative with acitin (-), CK7 (-), CD3 (-). Adenomatoid tumor is a rare benign neoplasm that should be added in the differential diagnosis of any adrenal tumor occurring in adrenal gland. The histological and immunohistochemical profiles of this adrenal adenomatoid tumor are very supportive in reaching the diagnosis of this benign tumor of a mesothelial cell origin, helping to avoid invasive treatment

Bladder mucinous adenocarcinoma as a diagnostic challenge: a case report

Primary bladder adenocarcinoma (PBA), especially the mucinous subtype, is a rare cancer that represents less than 2% of all bladder malignancies. Overlapping histopathological and immunohistochemical (IHC) features of PBA with metastatic colonic adenocarcinomas (MCA) make the final diagnosis very hard. We presented a 75-year-old woman presenting with hematuria and severe anemia in the last two weeks. The abdominal computed tomography scan showed a tumor-sized 2x2 cm right to the bladder dome. The patient underwent partial cystectomy without postoperative complication. The histopathologic and IHC showed mucinous adenocarcinoma and could not distinguish between the PBA from MCA. Investigations to exclude MCA revealed no other primary malignant site and suggested PBA. In conclusion, mucinous PBA requires ruling out any possibility of a metastatic lesion that could arise from other organs. Treatment should be considered an individual approach based on the tumor location and size, the patient´s age, general condition, and comorbidities

The role of inflammation and apoptosis in cyclosporine A – induced gingival overgrowth

Cyclosporin A(CsA) - induced gingival overgrowth(GO) is a current problem of tissue-specific mechanism which is still incompletely explained. The apoptotic process has been of particular interest like a new concept in the etiology of this unwanted effect. The aim of our study was to detect the level of apoptosis, expression bcl-2 and p53, associated with the different dosis of CsA. in gingival stroma. A cohort of 84 kidney transplant recipients was divided into four subgroups based on average daily dose of therapeutically applied CsA (Ne-oral®), (100 mg, 125 mg, 150 mg and 175 mg). The control group consisted of 21 patients, clinically diagnosed with periodontitis, who were not subjected to any medicamentous treatment causing gingival overgrowth. The following indexes were analyzed: plaque index (PI), index of gingival inflammation (GI) according to Loe-Silnes, and gingival overgrowth index (GOI) according to MacGaw et al. The tissue samples were subjected to a semiquantitative analysis to detect apoptotical cells and imunohistochemically stained to detect the expression of the bcl-2 and p53 proteins. The difference in percentage of apoptotic cells between the group taking 175mg and other subgroups, as well as the control group was statistically significant (p<0.05). There was a significant difference in percentage of expression bcl-2 between the 175 mg group compared to the other three subgroups and the control (p=0.001). However, a statistically significant positive correlation between the medicament dose, p53, apoptosis, and bcl-2 was registered (p<0.05). Inflammation plays the most important role in the induction of apoptosis and proliferation in gingival tissues

High expression of CD133 – stem cell marker for prediction of clinically agressive type of colorectal cancer

Background: Colorectal cancer (CRC) is one of the most common malignancies in the world. The cancer stem cell (CSC) markers are associated with aggressive cancer types and poor prognosis. The objective of the study was to evaluate the CD133 expression and to correlate it with clinicopathological features in patients with CRC. Material and Methods: Our study included ninety patients with CRC who underwent curative surgical resection from 2012 to 2017 at the University Clinic for Digestive Surgery, Skopje, North Macedonia. Tumor samples were first analyzed with standard histopathological methods and then the CD133 expression was investigated immunohistochemically. The level of expression of CD133 was classified semiquantitatively. Low positivity was defined as positive immunoreactivity in <50% of tumor glands, and high positivity was defined as positive immunoreactivity in ≥50% of tumor glands. Furthermore, clinicopathological features of patients were retrospectively reviewed. Results: High expression of CD133 was found in 47.8% of patients’ CRC samples. In 69.6% of patients with metastatic lesions in visceral organs we found high expression of CD133. We found statistically significant differences in the expression of CD133 between patients with and without visceral metastatic lesions (P = 0.0153), between patients with a different T category (P = 0.0119), N status (P = 0.0066) and grade (G) (P = 0.0115). Our results showed that the stage of disease has the greatest impact on expression of CD133 (P < 0.00001). Conclusion: High expression of CD133 is a useful marker for prediction of the clinically aggressive type of CRC and can be routinely implemented in standard pathohistological diagnostics

Wysoka ekspresja CD133 – markera komórek macierzystych do predykcji agresywnego klinicznie typu raka jelita grubego

Wstęp: Rak jelita grubego (RJG) jest jednym z najczęstszych nowotworów złośliwych na świecie. Markery komórek macierzystych raka (KMR) wiążą się z agresywnymi typami nowotworów i złym rokowaniem. Cel: Celem pracy była ocena ekspresji CD133 i skorelowanie jej z przyszłością kliniczno-patologiczną u pacjentów z RJG. Materiał i metody: Badaniami objęto dziewięćdziesięciu pacjentów z RJG, którzy w latach 2012–2017 poddali się leczniczej resekcji chirurgicznej w Klinice Uniwersyteckiej Chirurgii Trawiennej w Skopje, w Macedonii Północnej. Próbki guza analizowano najpierw standardowymi metodami histopatologicznymi, a następnie badano immunohistochemicznie ekspresję CD133. Poziom ekspresji CD133 został sklasyfikowany półilościowo. Niską wartość dodatnią zdefiniowano jako dodatnią immunoreaktywność w <50% gruczołów z guzem, a wysoką wartość dodatnią jako dodatnią immunoreaktywność w ≥50% gruczołów z guzem. Ponadto dokonano retrospektywnego przeglądu kliniczno-patologicznego przyszłości pacjentów. Wyniki: Wysoką ekspresję CD133 stwierdzono w 47,8% w próbkach RJG pacjentów. U 69,6% osób z przerzutami w narządach trzewnych zaobserwowano wysoką ekspresję CD133. Stwierdzono istotne statystycznie różnice w ekspresji CD133 między: pacjentami z i bez zmian przerzutowych trzewnych (p = 0, 0153), pacjentami z różnymi kategoriami T (p = 0,0119), stanem N (p = 0,0066) i różnicowaniem G (p = 0,0115). Nasze wyniki wykazały, że największy wpływ na ekspresję CD133 ma stadium choroby (p < 0,00001). Wnioski: Wysoka ekspresja CD133 jest przydatnym markerem do przewidywania klinicznie agresywnego typu RJG i może być rutynowo stosowana w standardowej diagnostyce patologicznej