Primary hepatic leiomyosarcoma: A case report and literature review.

BACKGROUND: Primary hepatic leiomyosarcoma (PHL) is a rare tumor with a very low incidence of about 0.2%. CASE SUMMARY: A 48-year-old diabetic, hypertensive, and morbidly obese female patient presented with a history of abdominal pain and weight loss for 2 mo. She had no history of fever, jaundice, or other liver disease(s). Clinical examination revealed a palpable mass in the epigastrium. Imaging evaluation with a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis revealed an ill-defined enhancing hyper vascular hepatic mass of 9.9 cm × 7.8 cm occupying the left hepatic lobe with evidence of central necrosis, compression effect on the left hepatic vein, and partial wash-out on delayed images. On further workup, the maximum standardized uptake value on positron emission computed tomography scan was 6.4, which was suggestive of malignancy. The remaining part of the liver was normal without any evidence of cirrhosis. Ultrasound-guided biopsy of the mass showed smooth muscle neoplasm suggestive of leiomyosarcoma. After optimization for co-morbidities, an extended left hepatectomy was planned in a multidisciplinary team meeting. On intraoperative ultrasound, the left hepatic lobe was entirely replaced by a large tumor extending to the caudate lobe with a compression effect on the middle and left hepatic veins. Final histopathology showed nodular and whorled white tumor comprised of spindled/fascicular cells with moderate to severe pleomorphism and focal necrosis. The mitotic index was greater than 20 mitoses per 10 high-power fields. The resection margins were free of tumor. Immunohistochemistry (IHC) depicted a desmin-positive/ caldesmon-negative/discovered on gastrointestinal stromal tumor 1-negative/ cluster of differentiation 117-negative profile, confirming the definitive diagnosis as PHL. CONCLUSION: This case report highlights the rare malignant mesenchymal hepatic tumor. To confirm PHL diagnosis, one requires peculiar histopathological findings with ancillary IHC confirmation. Management options include adequate/complete surgical resection followed by chemotherapy and/or radiotherapy.

Renal endometriosis: A benign disease with malignant presentation.

Endometriosis is a common gynecological disorder in which endometrial tissue is located outside the uterine cavity. Urinary tract involvement by endometriosis is uncommon. Renal endometriosis is a rare disorder and is not evident on imaging. It may be misdiagnosed as malignant disease and patients may undergo invasive procedures for it. We report a case of a young lady who presented with symptoms typical for renal mass, and was diagnosed as a tumor on imaging. Patient underwent radical surgery and histology revealed renal endometriosis.

Presentation of Acute Lymphoblastic Lymphoma and Colorectal Carcinoma in the Context of Constitutional Mismatch Repair Deficiency Syndrome: A Case Report with Literature Review.

Introduction: Constitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessive disease carrying an increased risk of cancers (paediatric tumours of central nervous system, haematolymphoid malignancies along with gastrointestinal (GI) cancer(s), which are usually seen in the second and third decades), leading to syndromic presentation. Causal mutations are detected in DNA mismatch repair (MMR) genes, including MLH1, PMS2, MSH2 and MSH6 that are also known for their established role in Lynch syndrome. We describe a case of CMMRD with an earlier (first decade of life) presentation of mediastinal acute lymphoblastic lymphoma and colorectal malignancy. Case Presentation: A 5-year-old boy presented with respiratory complaints, bilateral cervical lymphadenopathy, multiple café-au-lait macules (CALMs) on the lower back and history of parental consanguinity with the death of three sisters due to brain tumour within 6 months of diagnosis. Computerised tomographic scan chest revealed a huge mediastinal mass. The patient underwent a trucut biopsy of the mass. The results were significant for a pre-T-cell acute lymphoblastic lymphoma. Suspicion of CMMRD was raised based on a combination of factors described above. A panel of MMR proteins was applied on the biopsy tissue that revealed loss of nuclear expression of MLH1 and PMS2 immunostaining in tumour cells with positive external controls. While on maintenance therapy for lymphoma, about a year later, the patient developed subacute intestinal obstruction due to a stenosing polypoidal circumferential tumour in the mid-sigmoid colon found on flexible sigmoidoscopy that was followed by endoscopic biopsies and insertion of a fully covered self-expanding metallic adult biliary stent with a diameter of 10 mm and length of 6 cm leading to immediate relief of obstruction. Biopsies revealed adenocarcinoma with neuroendocrine differentiation. Metastatic tumour deposits were seen in the omentum, anterior abdominal wall and the left peritoneal wall. Practical Implications: Earlier (first decade) presentation of GI malignancy warrants that an earlier screening through radiological scans for any possible tumours and MMR protein expression analysis (loss in tumour plus normal non-tumour cells) are essential in patients having CALMs and family history of paediatric tumours.

Amelioration of airway inflammation and pulmonary edema by Teucrium stocksianum via attenuation of pro-inflammatory cytokines and up-regulation of AQP1 and AQP5.

Current study investigates the immunomodulatory effects of T. stocksianum using mouse model of ovalbumin (OVA)-induced allergic asthma. The mice were treated with methanolic extract, n-hexane, and ethyl acetate fractions for consecutive 7 days along with intranasal challenge. The mRNA expression levels of interleukin-4 (IL-4), IL-5, Aquaporin-1 (AQP1) and Aquaporin-5 (AQP5) were evaluated using reverse transcription polymerase chain reaction. The data showed that T. stocksianum significantly reduced airway inflammation as indicated by reduced inflammatory cell infiltration in lungs, and attenuated total and differential leukocyte counts both in blood and BALF. Expression levels of pro-inflammatory IL-4 and IL-5 in lungs were also found significantly reduced. T. stocksianum significantly reduced pulmonary edema as indicated by reduced lung wet/dry ratio and goblet cell hyperplasia. AQP1 and AQP5 expression levels were also found elevated in treatment groups. In conclusion, T. stocksianum possesses anti-asthmatic activity which may be attributed to reduction in IL-4 and IL-5 expression levels, and elevation in AQP1 and AQP5 expression levels.

Amelioration of allergic asthma by Ziziphora clinopodioides via upregulation of aquaporins and downregulation of IL4 and IL5.

Ziziphora clinopodioides has been frequently used as an anti asthmatic plant in traditional medication. Recent work explores the anti-asthmatic activity of Z. clinopodioides in allergen-induced asthmatic mice. Intraperitoneal sensitization followed by intranasal challenge were given with ovalbumin (allergen) to develop allergic asthma. Investigational groups of animals were administered with drug methylprednisolone (MP) (15 mg/kg body weight), n-hexane fraction, ethylacetate fraction, and methanolic extract of Z. clinopodioides extract (500 mg/kg b.w.) for successive 07 days. Hematoxyline and eosin (H&E) and periodic acid-Schiff (PAS) stains were used to evaluate histopathological parameters on lung tissues. As an index of lungs tissues edema, wet/dry weight ratio of lungs was determined. Evaluation of expression levels of AQP1, AQP5, IL4, and IL5 was conducted by using RT-PCR. The data exhibited that both Z. clinopodioides and MP attenuated differential and total leukocyte counts in hematological examination i.e. in BALF and blood. Treatment with Z. clinopodioides also caused suppression of inflammatory cell infiltration and expression levels of IL4 and IL5, the later could have caused attenuation of pulmonary inflammation. The study also found decline in lung wet/dry ratio and goblet cellh hyperplasia in treated groups which indicates amelioration of lung edema. Treatment with Z. clinopodioides significantly increased the expression levels of aquaporin-1 and -5, which could have led to reduction in lung edema. The treatment with MP showed comparable results to Z. clinopodioides. Current investigation revealed that Z. clinopodioides possessed anti-asthmatic property which might be accredited to upregulagted AQP1 and AQP5 levels and downregulated IL4 and IL5 levels.

Ziziphora clinopodioides ameliorated rheumatoid arthritis and inflammatory paw edema in different models of acute and chronic inflammation.

Ziziphora clinopodioides has been used in traditional medicine for its anti-inflammatory properties. Current study is believed to first time report the potential of Z. clinopodioides extracts to ameliorate joint inflammation using model of chronic joint inflammation (FCA-induced rheumatoid arthritis). The study further investigates the effects on joint inflammation using acute inflammatory paw edema models. The anti-inflammatory effects were also supported by using xylene-induced ear edema model. Results showed that Z. clinopodioides significantly ameliorated rheumatoid arthritis as indicated by the inhibition of arthritic development and paw edema. Histopathological examination showed significant attenuation in pannus formation, bone erosion, and joint inflammation. Treatment with the plant extracts also nearly normalized counts of RBCs, platelets, and total leukocytes along with hemoglobin (Hb) content. Biochemical analysis (AST, ALT, urea, and creatinine) showed that plant extracts did not possess hepatotoxic or nephrotoxic effects. Water displacement plethysmometric analysis showed that Z. clinopodioides significantly attenuated carrageenan-induced paw edema. To evaluate the mechanism, anti-inflammatory effects were further evaluated using histamine- and serotonin-induced inflammatory paw edema models. Z. clinopodioides significantly suppressed paw edema induced by both histamine and serotonin, and also caused the inhibition of xylene-induced ear edema. This suggested the inhibition of autacoids as one of the mechanisms of anti-inflammatory effects of plant. GC-MS analysis showed that the plant is rich in essential oils, including terpenoids, esters, alcohols, furans, cyclic ketones, epoxides, oxanes, and acyclic hydrocarbons. In conclusion, current study demonstrated that Z. clinopodioides possessed significant anti-arthritic and anti-inflammatory properties which might be attributed to the inhibition of autacoids.