A review of hirschsprung's disease in Hospital Universiti Sains Malaysia 1999-2004

Introduction Hirschsprung' s disease is a developmental disorder of the enteric nervous system causing congenital megacolon and the commonest cause of intestinal obstruction in the neonatal period. Being the only Pediatric surgical unit until the end of 2005 Hospital USM undertook all the references for Hirschsprung's disease in the upper east coast region of Malaysia. The treatment of the disease started when a patient is suspected of having the disease clinically. The patient would receive rectal irrigation until the diagnosis is confirmed by rectal biopsy. When the diagnosis is confirmed the child would have a colostomy created. When the child reached about one year old or weight of 10 kilograms, the definitive procedure would be done. Until 2004, the procedure of choice in the hospital was Duhamel procedure. After the definitive procedure, the colostomy would be closed. Objective: The objective of the study is to review the children presenting with Hirschsprung's disease to the hospital and described the demographics, mode and age of presentations, diagnosis, operative treatment, complications and outcome of the patients. Methodology: The study was retrospective review study of the patients who were diagnosed and had their definitive pull-through procedure in the hospital during a period of 5 years (from February 1999 - February 2004). Patients who defaulted prior to the definitive procedure were excluded. Results: There were 94 patients whose data was available for the review. There were 4: 1 male to female ratio with age of presentation ranging from I day old and I3 years old with 73.4% presented within the age of 3 months. The most common of clinical presentation was abdominal distension (87.2%) followed by vomiting (61.7%), poor feeding (55.3%), delayed passage of meconium (47.9%) and chronic constipation (46.8%). Seventeen percent (I 7%) of patients had other associated anomalies and 3.2% had a family history. Sixty three percent (63.4%) were diagnosed with rectal suction biopsy. Ninety five percent (95.7%) of patients had Duhamel type of pull-through procedure with 3 had transanal pull-through and only one who had Soave. There was a mortality recorded for the study and it was attributed to enterocolitis. The patient was also having Down syndrome and congenital heart disease. In the follow up period an average of 74% were free of complications of constipation, enterocolitis, incontinent, redo operation. There was I 0.6% reoperation rate. Five patients needed a complete redo of their definitive procedures. Conclusion The demographics finding of the study is quite similar to the patterns in the other parts of the world. The presenting symptoms were also quite similar to other studies. The definitive procedure of choice during the study time was Duhamel operation. The operative outcome was safe and acceptable

A rare case of non-neutropaenic typhlitis in immunocompetent patient – case report

Introduction: Typhlitis is necrotizing inflammation of the caecum manifested by febrile right lower quadrant abdominal pain. All cases in literature have been described in immunocompromised such patients with neutropenia, haematological or solid malignancies, and following immunosuppressive therapy. We believe this case represents an example of typhlitis in non-immunocompromised patient, among the first such case reported. Clinical case: We would like to highlight a case of a 49-year-old Malay lady, with unremarkable clinical background. She initially presented with sudden onset of progressively worsening right lower abdominal pain associated with high grade fever and tachycardia. Abdominal examinations revealed a palpable mass and localised tenderness in the right iliac fossa. Blood investigations showed leucocytosis without any significant neutropenia. Ultrasound showed thickening of surrounding mesenteric fat with an irregular tip appendicolith suggested a diagnosis of perforated appendix. Intraoperatively, revealed an inflammed pre-ileal appendix and perforated caecum 3cm from the base of the appendix. Her post-operative recovery was uneventful. Pathological findings: Gross examinations of the specimen showed an acutely inflammed appendix with a suspicious area of perforation noted at the caecum measuring 10mm with surrounding ischaemic areas covered with slough and grossly oedematous. Histopathologically, there is acute appendicitis with acute perforated caecitis or otherwise known as typhlitis. Conclusion: Typhlitis may present in immunocompetent patients and should be considered in the differential diagnosis of patients exhibiting febrile right lower quadrant abdominal distress. Early and appropriate diagnosis allows management at an early stage and prevents complications and mortality

Back pain as first presentation of Hepatocellular Carcinoma

Hepatocellular Carcinoma (HCC) is known to be one of the leading causes of deaths globally. In Malaysia HCC is known to be the eight most common cancer in both genders and the fifth most common cancer for males. The etiological factors associated with HCC are chronic Hepatitis B or C viral infection, liver cirrhosis and nonalcoholic fatty liver disease. Patients with HCC usually present with right upper quadrant pain, jaundice, loss of weight, and a palpable mass over the right hypochondrium. This case report will describe and discuss about the diagnosis of HCC in a patient with an atypical presentation of back pain which was confirmed with supportive findings of CT scan and MRI liver.peer-reviewe

Survival probabilities and prognostic factors of pregnancy-associated breast cancer in Malaysian women

Objective Pregnancy-associated breast cancer (PABC) is a rare cancer. This study aimed to determine the survival probabilities and prognostic factors in patients with PABC. Methods A retrospective cohort study was conducted in two tertiary care hospitals in Kota Bharu. We included all patients with breast cancer who were diagnosed by histopathology while pregnant or within 2 years post-partum from 2001 through 2020. We matched patients with PABC to non-pregnant patients with breast cancer by age and year of diagnosis. The data were analyzed using Cox proportional hazard regression. Results A total of 35 cases of PABC and 70 non-PABC controls were recruited. The 3-year, 5-year, and 10-year survival probabilities for patients with PABC were 58.6%, 47.54%, and 38.03%, respectively. The patients with PABC had a non-significant difference in survival probabilities compared with non-PABC patients. The significant prognostic factors of PABC were age (adjusted hazard ratio [aHR], 0.91; 95% confidence interval [CI], 0.86–0.96; P=0.001), advanced stage of cancer (aHR, 9.97; 95% CI, 3.96–25.2; P<0.001), and no surgery (aHR, 3.16; 95% CI, 1.01–9.85; P=0.047). Pregnancy was not found to be an independent factor in the prognosis of PABC (aHR, 0.72; 95% CI, 0.39–1.28; P=0.266). Conclusion Women diagnosed with PABC had similar survival probabilities compared with non-PABC patients. Pregnancy was not an independent prognostic factor for breast cancer. This information can be useful when women with breast cancer are counseled and supported with the option of beginning treatment with pregnancy continuation

A harmless evil: giant fungating benign breast mass in an adolescent mimicking malignancy - case report

Introduction: Juvenile giant fibroadenoma is a rare type of fibroadenoma characterized by rapid growth of a breast tumor in an adolescent. Benign in nature, they rarely present as fungating and ulcerating tumors. Benign tumors masquerading as malignancies are surgical conundrums. No co nsensus exists yet on the management of these cases. We aim to discuss the dilemma in managing a bleeding, fungating giant fibroadenoma in an adolescent female and highlight risks of alternative therapies. Presentation of case: A 19-year-old lady presented with a bleeding, fungating breast mass worsened with topical herbal concoction. Examination revealed a 10 × 15 cm fungating breast mass that obliterated her nipple- areolar complex (NAC). Computed Tomography (CT) scan reported a huge heterogeneously enhancing mass 10.6 — 14.5 — 15.1 cm with loss of normal fat plane with the overlying skin but a clear fat plane with the pectoralis muscle posteriorly. Discussion: Giant breast masses that fungate and ulcerate usually indicate a sinister pathology. Traditional remedies have been reported to exacerbate growth. In cases where most of the breast parenchyma and NAC has been destroyed, it is no longer possible to proceed with breast conserving techniques. Breast reconstruction is crucial in adolescents and should be tailored to the patient's existing breast size as well as body habitus. Conclusion: In juvenile giant fibroadenomas where breast parenchyma and NAC has been destroyed, breast reconstruction is the goal. The lack of consensus in both diagnosis and management further compounds the difficulty in dealing with this sensitive population. Awareness needs to be raised regarding negative effects related to traditional medicine

Association of serum vitamin D level and vitamin D receptor expression among newly diagnosed breast cancer

There is a mushrooming interest in the anti-carcinogenic property of vitamin D. However, many researches reported a conflicting result in the association of vitamin D levels to certain types of cancer. This study was designed to assess the association between vitamin D and vitamin D receptor (VDR) expression with breast cancer. This case-control study, carried out at Hospital Universiti Sains Malaysia, Kelantan, involved 69 newly diagnosed breast cancer patients and 73 healthy volunteers. Serum 25(OH)D was taken and compared between 2 groups. VDR expression in patients’ breast tissue samples was determined by immunohistochemical staining method using anti-VDR antibody. 85.5% of breast cancer patients and 97.3% of healthy control were vitamin D insufficient with a mean (SD) of 13.36 (6.96) ng/mL and 13.05 (3.71) ng/mL, respectively, and the difference was not statistically significant. VDR expression showed cytoplasmic positivity in 75.4% of breast cancer tissue, followed by both cytoplasmic and nuclear positivity in 21.5% and complete absence in 3%. There was no significant association between VDR expression and hormone receptor status. In conclusion, there was a high prevalence of vitamin D deficiency among breast cancer and healthy volunteers in our study. There was no significant association between breast cancer and vitamin D. The VDR expression in breast cancer cells showed high cytoplasmic localization

Circulating neonatal Nav1.5 (nNav1.5) antigen and anti-nNav1.5 antibodies as potential biomarkers for breast cancer metastasis

Neonatal Nav1.5 (nNav1.5) has been known to potentiate breast cancer (BCa) metastasis. The detection of anti-nNav1.5 antibodies (anti-nNav1.5-Ab) reflects the immunogenicity of nNav1.5. However, the presences of circulating nNav1.5 antigen and anti-nNav1.5-Ab in the context of BCa metastasis have not been explored yet. Therefore, the study has attempted to conduct such an investigation using both blood samples from 4T1 orthotopic mice and BCa patients. In the preclinical study, forty female BALB/c mice were divided into three groups: 4T1 orthotopic BCa mice (n=17), control mice (n=20) and positive control mice (n=3). After tumour development, the mice were sacrificed to obtain target organs, whole blood, and serum. Histopathology, cytokine analyses, real-time PCR, and indirect ELISA were performed. Histopathology and cytokine analyses showed the establishment of metastasis in 4T1 orthotopic mice. The concentration of vascular endothelial growth factor (VEGF) was significantly higher in the 4T1 orthotopic mice (P<0.0001****). Circulating nNav1.5 antigen and anti-nNav1.5-Ab were detected in 4T1 orthotopic mice, using real-time PCR and indirect ELISA, respectively. Furthermore, there was an inverse relationship between anti-nNav1.5-Ab and the total metastatic foci (P=0.0485*, r=-0.7306). In the clinical study, 32 BCa patients were grouped based on their stages: early-invasive (n=15) and advanced (n=17) stages. Approximately 3 mL of blood was withdrawn, and only indirect ELISA was conducted. The clinical study showed that BCa patients of advanced-stages portrayed higher expression of anti-nNav1.5-Ab compared to early stages of BCa (P =0.0110*). In conclusion, the detection of nNav1.5 antigen and anti-nNav1.5-Ab was consistent with the presence of BCa metastasis

Synchronous Breast and Cervical Carcinoma: A Genetic Point of View

Breast carcinoma is the most common cancer of women in Malaysia. The most common sites of metastasis are the lung, liver, bone and brain. A 45-year-old lady was diagnosed with left invasive breast carcinoma stage IV (T4cN1M1) with axillary lymph nodes and lung metastasis. She was noted to have a cervical mass through imaging, and biopsy showed CIN III. Post chemotherapy, the patient underwent left simple mastectomy with examination under anaesthesia of the cervix, cystoscopy and staging. The cervical histopathological examination (HPE) showed squamous cell carcinoma, and clinical staging was 2A. The breast tissue HPE showed invasive carcinoma with triple receptors positivity. The patient was given tamoxifen and put on concurrent chemoradiotherapy (CCRT) for the cervical cancer. The management of each pathology of this patient involved a multi-disciplinary team that included surgeons, oncologists, gynaecologists, pathologists and radiologists. Due to the complexity of the case with two concurrent cancers, the gene expression profiles may help predict the patient&rsquo;s clinical outcome

Feasibility analysis of treating breast cancer patients with breast-conserving surgery via a periareolar incision combined with non-lipolytic suspension-type mastoscopy

Abstract The purpose is to analyze and compare postoperative recovery and complication incidence between a periareolar incision combined with Suspension-type Mastoscopic Axillary Lymph Node Dissection (SMALND) and traditional inflated Mastoscopic Axillary Lymph Node Dissection (MALND). This was a randomized trial conducted from June 1, 2020, to April 30, 2022, in the Department of Second Breast Surgery, Shengjing Hospital of China Medical University, and the Department of Thyroid and Breast Surgery, Zibo Central Hospital, in accordance with the criteria of inclusion and exclusion. Overall, 126 patients diagnosed and treated for early-stage breast cancer were selected to undergo periareolar-incision breast-conserving surgery. Those patients who underwent periareolar-incision surgery combined with SMALND formed the observation group (SMALND Group), while those who underwent periareolar-incision surgery combined with traditional inflation became MALND Group. In the two groups, paired data “t” was used to examine, analyze, and compare the postoperative daily drainage volume and drain removal time, while paired data “χ2” was used to examine, analyze, and compare the incidences of postoperative upper limb edema and paresthesia. There were 64 cases in the SMALND Group and 62 cases in the MALND Group. Between the two clusters, no differences were found in age, clinical staging, BMI, and breast cancer classification (P > 0.05). The intraoperative surgery time of the SMALND Group was 43.37 ± 6.27 min while that of the MALND Group was longer: 45.72 ± 4.25 min (P < 0.05). The intraoperative hemorrhage volume of the SMALND Group was 88.33 ± 16.79 ml, less than that of the MALND Group: 96.76 ± 26.85 ml (P < 0.05). The postoperative axillary mean daily drainage volume of the SMALND Group was 38.17 ± 5.55 ml, less than that of the MALND Group: 40.72 ± 7.25 ml (P < 0.05). The drain removal time of the SMALND Group was 7.50 ± 1.60, less than that of the MALND Group: 9.00 ± 1.80 (P < 0.05). The upper limb edema incidence rate of the SMALND Group was 3.12% (2/64) and had no obvious difference from the MALND Group, which was 4.83% (3/62) (P = 0.62). The paresthesia incidence rate of the SMALND Group was 18.75% (12/64), while that of the MALND Group was 17.7% (11/62), without an obvious difference (P = 0.88). For axillary lymph node dissection, the use of non-lipolytic suspension-type mastoscopy has reduced the intraoperative hemorrhage volume of patients, shortened surgery time and postoperative recovery time, saved treatment expenses for patients, and avoided complications such as hypercapnia and subcutaneous emphysema caused by traditional inflated mastoscopic surgery. Moreover, it has not increased the incidence of postoperative upper limb edema and paresthesia, supporting its safety and effectiveness

The Unique Biology behind the Early Onset of Breast Cancer

Breast cancer commonly affects women of older age; however, in developing countries, up to 20% of breast cancer cases present in young women (younger than 40 years as defined by oncology literature). Breast cancer in young women is often defined to be aggressive in nature, usually of high histological grade at the time of diagnosis and negative for endocrine receptors with poor overall survival rate. Several researchers have attributed this aggressive nature to a hidden unique biology. However, findings in this aspect remain controversial. Thus, in this article, we aimed to review published work addressing somatic mutations, chromosome copy number variants, single nucleotide polymorphisms, differential gene expression, microRNAs and gene methylation profile of early-onset breast cancer, as well as its altered pathways resulting from those aberrations. Distinct biology behind early-onset of breast cancer was clear among estrogen receptor-positive and sporadic cases. However, further research is needed to determine and validate specific novel markers, which may help in customizing therapy for this group of patients