Primary Cutaneous Melanoma in a Tertiary Hospital: A Retrospective Study.

BACKGROUND: Malignant melanoma, the most aggressive form of skin cancer worldwide is much often talked about in the western world and in Caucasian population as it is seen to be a rare disease in South Asians. This study aims to provide a better assessment on the spectrum of cutaneous melanomas in our context. METHODS: This was a retrospective, 11-year hospital-based study done in the Department of Pathology, Tribhuvan University Teaching Hospital. The data collected included age, sex, anatomical site, Breslow thickness in millimeter, Clark's level of invasion, presence of ulceration, Tumor infiltrating lymphocytes and melanoma subtypes. The anova test was used to compare the relationship between age and staging, whereas pearson's chi square test was used to determine the relationships of sex and histopathological subtype with staging. RESULTS: Out of total, 44 cases of primary cutaneous melanoma were seen, 23 (52.27%) were males and 21 (47.27%) were females. The mean age was 61.29 year with the majority in the age groups 61-70 and 71-80 comprising 11 cases each (25%). Lower extremity was the most frequent site (23 cases, 52.27 %). The largest group (18 cases) was composed of Nodular melanoma, followed by acral melanoma (17 cases). Nodular melanoma tended to occur at a higher stage than other types. Age and sex showed no correlation with staging. A significant association was found with histopathological type. CONCLUSIONS: Primary melanoma is a commonly encountered malignancy. It is commomly appreciated equally among both gender among elderly populations. Nodular melanoma present late and is the commonest variety with lower extremity being the commonest site Keywords: Breslow thickness; clark's level; cutaneous melanoma; nodular melanoma.

Multiple Cavernous Haemangioma of Orbit and Cranium: A Case Report.

A 32-year-old male presented with painless proptosis and diminution of vision in left eye. Imaging shows multiple well-defined masses, suggestive of cavernous haemangioma, in orbit and cranium with adjoining bones being thickened with cystic spaces. Histopathology proved the diagnosis. Cavernous haemangioma usually presents as a solitary intraconal and sometimes extraconal mass with vision usually preserved unless it extends to the apical portion. Here we report a rare case of multiple simultaneous locations of cavernous haemangiomas in orbit and cranium with significant diminution of vision.

Primary Duodenal Adenocarcinoma: Uncommon Tumor, Uncommon Presentation. A Case Report and Review of the Literature.

INTRODUCTION: The symptoms of primary duodenal adenocarcinoma, which is a rare but aggressive tumor, are vague and nonspecific and often result in a delayed diagnosis or misdiagnosis. This results in a tumor being diagnosed at an advanced stage when it becomes unresectable secondary to local and distant spread. CASE PRESENTATION: A 64-year-old Nepalese female presented to our hospital with epigastric pain, anorexia, and significant weight loss that developed over two-and-a-half months. Upper gastrointestinal endoscopy showed an ulceroproliferative growth in the first part of the duodenum with no features of duodenal stenosis. Contrast-enhanced computed tomography of the abdomen revealed heterogeneously enhancing, circumferential, asymmetrical thickening in the first part of the duodenum and multiple liver metastases. Biopsy of the mass revealed features suggestive of moderately differentiated adenocarcinoma of the duodenum. She was managed with palliative care during her hospital stay. The unique presentation in our case was that the tumor did not cause stenosis and the patient could consume food till the last day of her life. CONCLUSION: In patients with primary duodenal adenocarcinoma, the non-stenotic lesion is also a possibility. Clinicians should always maintain a high degree of suspicion to avoid the delay in diagnosis or misdiagnosis.

Diffuse Alveolar Haemorrhage as Initial Presentation of Systemic Lupus Erythematosus: A Case Report.

Diffuse alveolar hemorrhage results from accumulation of red blood cells in the alveolar space originating from alveolar capillaries. Alveolar hemorrhage in Systemic Lupus Erythematosus is rare but catastrophic and can rapidly progress to respiratory failure. We report a 22-year old lady who presented with dyspnoea on exertion, hemoptysis, bilateral leg swelling and oliguria. Diffuse alveolar hemorrhage was confirmed by bronchoalveolar lavage fluid analysis. Serologic tests and renal biopsy confirmed lupus nephritis. She was treated with systemic immunosuppressive therapy and plasma exchange, to which she had a favourable response. Lupus presenting as alveolar hemorrhage is rare which warrants prompt diagnosis and treatment to prevent complications. Keywords: Bronchoalveolar lavage; Case report; Diffuse alveolar hemorrhage; Systemic lupus erythematosus.

Doege-Potter Syndrome, cause of nonislet cell tumor hypoglycemia: the first case report from Nepal.

Doege-Potter syndrome (DPS), a paraneoplastic syndrome, presents as a hypoinsulinemic hypoglycemia from the ectopic secretion of insulin-like growth factor II from a solitary fibrous tumor which may be intrapleural or extrapleural in origin. We report a case of severe hypoglycemia in a 70-year old female initially admitted for resection of left sided solitary fibrous tumor of pleura. Investigation revealed true hypoglycemia, and DPS was diagnosed. The tumor was completely resected, after which no further hypoglycemic episodes were seen in 2 years follow-up. This is the first case of solitary fibrous tumor of pleura with DPS reported from Nepal.

Lipoblastoma: an unusual tumour of the left ventricle.

Lipoblastoma is a rare benign tumour of infancy originating from white foetal adipose tissue. Most commonly located in the extremities, intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer, with only one reported case. Herein, we present a 2-month old asymptomatic boy diagnosed with an echogenic mass in the left ventricular outflow tract. The patient underwent surgical excision and histopathological evaluation revealed a lipoblastoma.

Breast cancer in young women from a low risk population in Nepal.

BACKGROUND: The overall incidence of breast cancer in South Asian countries, including Nepal, is low compared to Western countries. However, the incidence of breast cancer among young women is relatively high. Breast cancer in such cases is characterized by a relatively unfavorable prognosis and unusual pathological features. The aim of this study was to investigate clinico-pathological and biological characteristics in younger breast cancer patients (<40 years) and compare these with their older counterparts. MATERIALS AND METHODS: Nine hundred and forty four consecutive female breast cancer patients, admitted to the Department of Surgery, Tribhuvan University Teaching Hospital, Kathmandu, Nepal between November 1997 and October 2012, were retrospectively analyzed. RESULTS: Out of the 944 female breast cancer patients, 263 (27.9%) were <40 years. The mean age was 34.6 ± 5.0 years among younger patients compared to 54.1 ± 9.9 for those ≥ 40 years. The mean age at menarche was also significantly lower (13.5 ± 1.5 vs 14.2 ± 1.5 years p=0.001) while the mean duration of symptoms was significantly longer (7.6 vs 6.5 months p=0.004). Family history of breast cancer was evident in 3.0% of the young women versus 0.3% in the older one. Mammography was performed less frequently in younger patients (59.7%), compared to older (74.4%), and was of diagnostic benefit in only 20% of younger patients compared to 85% of older ones. At diagnosis, the mean tumor diameter was significantly larger in young women (5.0 ± 2.5 vs 4.5 ± 2.4 cm, p=0.005). Axillary lymph nodes were positive in 73% of younger patients and 59% of older patients. In the younger group, the proportion of stage III or IV disease was higher (55.1% vs 47.1%, p ≤ 0.05). The proportion of breast conserving surgery was higher in young patients (25.1% vs 8.7%) and a higher proportion of younger patients receive neoadjuvant chemotherapy (9.9% vs 2.8%). The most common histological type was ductal carcinoma (93.1% vs 86%). The proportion of histological grade II or III was higher in younger patients (55.9% vs 24.5%). Similarly, in the younger group, lymphatic and vascular invasion was more common (63.2% vs 34.3% and 39.8% vs 25.4%, respectively). Patients in the younger age group exhibited lower estrogen and/ or progesterone receptor positivity (34.7% vs 49.8%). Although statistically not significant, the proportion of triple negative tumors in younger age group was higher (22.4% vs 13.6%). CONCLUSIONS: Breast cancer in young Nepalese women represents over one quarter of all female breast cancers, many being diagnosed at an advanced stage. Tumors in young women exhibit more aggressive biological features. Hence, breast cancer in young women is worth special attention for earlier detection.

Kikuchi-Fujimoto disease in Nepal: a study of 6 cases.

CONTEXT: Kikuchi-Fujimoto disease is a benign disorder, predominantly affecting young women with a predilection for cervical lymphadenopathy. Although the disease has been recognized worldwide, to our knowledge no cases have been reported previously from Nepal. OBJECTIVES: To determine the prevalence of Kikuchi-Fujimoto disease in Nepal and to analyze clinicopathologic features. METHODS: We reviewed 6 cases of Kikuchi-Fujimoto disease recorded at 3 different hospitals in Nepal during a period from June 1998 to June 2002. Clinical data and histopathology are presented. RESULTS: This study included 5 females and 1 boy, aged 13 to 32 years. These patients presented with prolonged fever and lymphadenopathy. The 5 female patients had cervical lymphadenopathy, and the boy had axillary lymphadenopathy. Complete blood counts revealed raised erythrocyte sedimentation rates in all patients and anemia in 2 patients. The size of excised lymph nodes (in greatest dimension) ranged from 1.5 to 5 cm. Typical histologic features were seen, namely, architectural effacement due to presence of pale nodular lymphohistiocytic foci with karyorrhectic debris, coagulation necrosis, eosinophilic debris, and absence of granulocytic infiltration. In a follow-up of the cases, disease recurrence was not found. CONCLUSION: Our study emphasizes that Kikuchi-Fujimoto disease should be considered as one of the differential diagnoses in patients with prolonged fever and cervical lymphadenopathy and that it should be differentiated from tuberculous lymphadenitis in regions where tuberculosis is prevalent.