Extra-gastrointestinal stromal tumor presenting as a huge peritoneal mass and mimicking as mesothelioma - A case report.

INTRODUCTION AND IMPORTANCE: Extra-gastrointestinal stromal tumors are group of soft tissue neoplasm, which originates outside the gastrointestinal tract comprising of less than 5% of the total gastrointestinal stromal tumors. CASE PRESENTATION: A 67 years old male came with a history of vague abdominal pain, discomfort and loss of appetite. Per abdominal examination showed a palpable firm mass which was filling both the flanks. Radiological imaging revealed a large abdomino-pelvic mass with central necrotic areas. Exploratory laparotomy was done and the mass was excised intact from the sigmoid mesocolon. Histopathological diagnosis was given as extra-gastrointestinal stromal tumors. CLINICAL DISCUSSION: Extra-gastrointestinal stromal tumor was first described by Miettinen et al. in 1999. The tumor can arise from the pleura, omentum, mesentery, retroperitoneum and prostate. The clinical presentation of the tumor depends on its location and the size of tumor. Patients with these tumors present with abdominal pain, followed by abdominal mass and distention. These tumors show pathological, immunohistochemical and molecular biological characters similar as that of gastrointestinal stromal tumor. CONCLUSION: Extra-gastrointestinal stromal tumor is a rare tumor and can reach to a considerable large size before presenting with clinical symptoms especially if the tumor arises from the mesocolon. IHC study plays an important role to reach to the final diagnosis as the tumor can mimic mesothelioma in routine staining.

A Misdiagnosed Case of Hypertrophic Gastropathy.

Hypertrophic gastropathy is a rare idiopathic hyperproliferative disorder which may present as Menetrier's disease (MD) characterized by foveolar hyperplasia in the gastric fundus and body. It is often accompanied by a severe loss of plasma proteins (including albumin) from the altered gastric mucosa. The disease occurs in two forms, a childhood form due to cytomegalovirus infection and an adult form attributed to overexpression of transforming growth factor-alpha (TGF-α). The most common symptoms include epigastric pain with fullness and vomiting and generalized peripheral edema with hypoalbuminemia. We present a case of 75-year-old female presenting with epigastric pain and vomiting. Upper gastrointestinal endoscopy and computed tomography scan revealed an irregular mucosal fold at the body and antrum and thickening of the stomach wall, respectively. Though the endoscopic gastric mucosal biopsy was nonspecific, the patient underwent partial gastrectomy due to clinicoradiological suspicion of carcinoma. On histopathology, the case was reported as hypertrophic gastropathy, consistent with MD. Though there is a strong clinical and radiological suspicion of malignancy in the hypertrophied gastric mucosa, MD should be one of the important differential diagnoses.

Hydatidosis of infratemporal fossa with proptosis - an unusual presentation: a case report and review of the literature.

BACKGROUND: Hydatid disease is one of the common zoonotic diseases caused by the larval stage of Echinococcus granulosus. It is endemic in sheep-raising and cattle-raising areas worldwide and humans are an accidental intermediate host following the ingestion of the larvae. Head and neck involvement of echinococcosis is a rare entity and involvement of the infratemporal region is extremely rare even in endemic areas. Only a few cases of hydatid cysts located in the infratemporal fossa have been reported in the literature. Moreover, extension of the hydatid cyst into the intraorbital region and infiltrating into the surrounding orbital bone is even rarer. CASE PRESENTATION: We present a case of a 65-year-old Gurung Nepalese woman with painless proptosis of her left eyeball of 2 months' duration with recent progressive diminution of vision for 15 days. Radiological findings showed a cystic mass in the left infratemporal fossa extending into the left orbit and involving the surrounding orbital bone. Surgical removal was carried out. On histopathological evaluation, it was reported as hydatid cyst infiltrating into the bone. She was prescribed albendazole and discharged after surgery. However, she was lost to follow up and returned after 15 months with recurrence and proptosis of the same eye. Repeat excision of the lesion was carried out and postoperatively she was administered tablet albendazole. She was found to be disease free after 6 months of follow up. CONCLUSIONS: Clinical and radiological findings are important but may not be sufficient in the preoperative diagnosis of hydatid disease especially if rare sites are involved. Proptosis may be seen in several conditions and orbital or infratemporal hydatidosis, although rare, should be considered a differential diagnosis.

Clinicohematological Study of Pancytopenia in a Tertiary Care Hospital of Western Region of Nepal.

INTRODUCTION: Pancytopenia is a relatively common hematological entity and is a manifestation of many illnesses which can be life threatening at times. The severity of pancytopenia and the underlying pathology determine the management and prognosis. This study was conducted to evaluate hematological and bone marrow findings in patients presenting with pancytopenia. METHODS: A prospective observational study was conducted in Department of Pathology, Manipal College of Medical Sciences, Pokhara from January 2011 to December 2016. Clinical and hematological parameters including bone marrow aspiration and biopsy were evaluated in all patients who presented with pancytopenia. RESULTS: Among 138 cases studied, patients' age ranged from 2 to 82 years with a mean age of 43.95 years, and there was male predominance. Most of the patients presented with generalized weakness, pallor, dypnoea and fever. Hypoplastic marrow was seen in 38 (27.5%) cases, followed by 26 (18.8%) cases of megaloblastic anemia and 19 (13.76%) cases of acute leukemia. Other findings included one case each of hemophagocyosis, leishmaniasis, plasmodium vivex malaria and metastatic carcinoma. CONCLUSIONS: This study highlights that pancytopenia is a common hematological problem and that the study of detailed primary hematological investigations along with bone marrow study in patients with pancytopenia will help to identify the cause for further planning and management.

Squamous cell carcinoma arising in familial gingival fibromatosis-a rare case report.

Familial gingival fibromatosis is a rare hereditary condition due to chromosomal abnormality which can occur as an isolated disease or as part of a syndrome and has an incidence of 1:350 000. This condition leads to esthetic, functional, psychological and masticatory disturbance of the oral cavity. Here, we present a case of 21-year-old female with severe enlargement of gums in maxilla and mandible. Deciduous teeth were erupted at normal age but the permanent teeth in the oral cavity were not erupted. Her grandmother, father and younger sister were also affected with the same condition. Incisional biopsy from the chronic ulcerated gingiva showed squamous cell carcinoma. Patient was referred to other cancer institution, where metastases to lung, bone and lymph node was detected on CT scan. The diagnosis was made based on clinical examination, family history and histopathological examination.

Warty Carcinoma Penis: An Uncommon Variant.

Penile carcinoma frequency varies widely in different parts of the world and comprises 1-10% of all the malignancies in males. Majority of the cases of penile carcinoma are squamous cell carcinoma of penis comprising 60% to 70% of all cases. Warty carcinoma of penis is an unusual neoplasm and a variant of penile squamous cell carcinoma comprising 5%-10% of all the variants. The other histological variants include basaloid, verrucous, papillary, sarcomatous, mixed, and adenosquamous carcinoma. The various histological entities with an exophytic papillary lesions including warty carcinoma are together referred to as the "verruciform" group of neoplasms. The warty carcinoma has to be differentiated from these lesions and is typically distinguished by histological features of hyperkeratosis, arborescent papillomatosis, acanthosis, and prominent koilocytosis with nuclear pleomorphism. We present a case of 65-year-old male with growth measuring 6 × 4 cm in the penis who underwent total penectomy and was diagnosed as warty carcinoma penis.

Basal Cell Carcinoma in Cases with or without Xeroderma Pigmentosum.

INTRODUCTION: Basal cell carcinoma is the most common form of cancer in humans and comprises the vast majority of skin cancers. It predominantly affects fair-skinned individuals, and its incidence is rapidly increasing. The objective of the study is to identify the epidemiology, its topography and different histological subtypes of basal cell carcinoma in patients with or without Xeroderma Pigmentosum. METHODS: A cross-sectional descriptive study was conducted at Manipal Teaching Hospital, Pokhara from Jan 2009 to Dec 2016. Ethical approval was taken from MEMG/IRC/GA. The study included patients with a confirmed diagnosis of basal cell carcinoma irrespective of their age and sex. RESULTS: This study showed 77 individuals with 91 biopsies of BCC including 5 cases of Xeroderma Pigmentosum. The predominant histological subtype was nodular with 41 (53.94%) cases, followed by the 14 (18.42%) cases of pigmented and 10 (13.15%) cases baso-squamous subtype. The most frequent sites of involvement were the head and neck, with predominance in the nasal and orbital region. The mean age was 57.68 years but the basal cell carcinoma in cases of Xeroderma Pigmentosum was seen more in younger age groups. There were 43 (55.84 %) male patients and 34 (44.16 %) female patients with a male to female ratio of 1.26:1. CONCLUSIONS: Nodular and pigmented varieties were the most frequent subtypes with nose being the commonest site of involvement. Basal cell carcinomas in cases of Xeroderma Pigmentosum were noted in younger age group with multiple lesions.

Histopathological Analysis of Non-Neoplastic Superficial Lymphadenopathies.

Background Lymphadenopathies are the clinical manifestation of enlargement of lymph nodes which are a common occurrence and are processes of lymph nodes in response to a variety of exogenous and endogenous stimulants. The vast majority of enlarged lymph nodes are non- neoplastic. Objective The objective of the study is to analyze the spectrum of non-neoplastic lesions of superficial lymphadenopathies with their histomorphological features. Method This was a retrospective hospital based study done in the department of Pathology. All cases of superficial lymph node biopsies received during a period of 7 years were retrieved and divided into 2 broad categories: neoplastic and non-neoplastic. The latter group is the material for the present study. The diagnosis was made on morphological basis. Result Of all the 268 superficial lymph node biopsies, 25.4% (68 cases) were neoplastic and 74.6% (200 cases) were non-neoplastic. The non-neoplastic cases were further categorized into non-infectious 60.5% (121 cases) and infectious 39.5% (79 cases). Neck node (70%) was the most common site of involvement. There were 102 male and 98 female patients with male to female ratio 1.04:1. The age range of the patients was 2 years to 83 years with a mean of 27.16 years and most common being 11-20 years 29% (58 cases). Majority of the cases were non-specific reactive lymphadenitis 84.3% (102 cases) followed by tuberculosis 36.5% (73 cases). Conclusion The major cause for the superficial lymphadenopathy was found to be non-neoplastic etiology with slight male predominance. Reactive lymphadenitis was the leading cause unlike the studies done in other Asian and Tropical countries where tuberculosis was more common.

Ovarian Tumors: Pattern of Histomorphological Types- A 10 Years Study in a Tertiary Referral Center and Review of Literature.

Background Ovarian cancer accounts for 6% of all cancers in females. Among cancers of female genital tract, the incidence of ovarian cancers ranks below only carcinoma of the cervix and the endometrium. Objective To find the frequency of different types of histomorphological types and their association with age, side and size distribution. Method This study is a hospital based retrospective study carried out in the Department of Pathology, Manipal Teaching Hospital over a time period from Jan. 2006 to Sep. 2015. Specimens were received from the Manipal Teaching Hospital and other hospitals within the Himalayan valley of Pokhara. All the clinical and histolpathological data were retrieved from the departmental data bank and were analyzed. Result A total of 409 cases of ovarian tumors have been reported in the same period. Among them, 215 cases were of surface epithelial origin including 172 benign, 07 borderline and 36 malignant cases. Among the surface epithelial tumors, the commonest was serous cystadenoma (119 cases) followed by mucinous cyst adenoma (40 cases). There were 176 cases of tumors with germ cell origin which included 170 cases of teratoma, four dysgerminoma and one each case of choriocarcinoma and yolk sac tumor. Among seven metastatic tumors, one case was of Krukenberg tumor. Age range was 12 to 90 years and the size range from 3 to 36 cm. Conclusion In our study, we analyzed all the spectrum of ovarian tumors diagnosed on morphological bases and most of the findings were similar to other national and international studies. However, we have reported increased percentage of mucinous tumors and less sex cord stromal tumors.

14 years hospital based study on clinical and morphological spectrum of hydatid disease.

INTRODUCTION: Hydatid disease is endemic in sheep and cattle-raising areas worldwide. Its prevalence is high in Nepal. The study was carried out to determine the clinical, radiological and pathological presentations of hydatid disease. METHODS: This was a retrospective study of all hydatid disease cases reported in Department of Pathology, from August 1996 to July 2010. All the clinical, radiological and pathological data were collected and collated. RESULTS: A total of 51 cases of hydatidosis were studied. Patients presented with related symptoms in 47 cases, 92.16% with CI (84.78, 99.54) and asymptomatic in four cases, 7.84% with CI (0.46, 15.22). Involvement of liver and lung was found in 35 (68.63%) cases and 10 (19.61%) cases respectively. Involvement of other organs like kidney, pelvis and broad ligament were seen in 6 of the cases. Total 29 cases, 56.86% with CI (43.2, 70.46) had solitary cyst while rest of the cases had multiloculated cyst. All cases had radiological correlation and histopathological confirmation. CONCLUSIONS: Most cases presented with organ related vague symptoms, however it should be considered as a differential diagnosis especially in asymptomatic cases and cases with unusual sites. Imaging studies is useful in preoperative diagnosis and postoperative histopathology is confirmatory. A multicentric hospital based study will help to decrease the incidence.

Value of image-guided fine-needle aspiration cytology-a study of 500 cases.

Guided Fine-Needle Aspiration Cytology (FNAC) as the first line investigation is not only useful in diagnosis of space occupying lesions but can also help in choosing appropriate management. This technique is most useful in diagnosing metastasis but is also helpful in excluding malignancy in some cases. Aim of this study is to analyze the spectrum of cytological diagnosis, adequacy, and clinicoradiological correlation of guided FNAC. The study was carried out in the departments of pathology and radiology for a period from January 2000 to November 2011. All the data were retrieved from hospital medical record section and departmental data bank and were analyzed. We have performed a total of 500 FNACs, including 234 under CT guidance and 266 under USG guidance. Among the CT-guided FNACs, 87.6% (205 cases) had adequate material which included 152 malignant cases, 38 inflammatory cases and 15 benign cases. Among the cases under USG, 91.7% (244 cases) had adequate material comprising of 112 malignant cases, 44 inflammatory cases, and 88 benign lesions. It was noted that increased number of inadequate aspirate was from lesions with smaller size and increased depth. Out of total 264 malignant cases diagnosed on FNAC, 160 cases were identified as malignant on clinical and radiological findings. Guided FNAC is useful in deep seated lesions as well as in suspicious superficial lesions and is a safe diagnostic tool. If performed properly and correlated well with clinical and radiological findings, FNAC provides correct diagnosis in most cases.

Patterns of hematological and non-hematological malignancies in bone marrow in a tertiary care hospital in Nepal--11 years study.

Developing countries bear more than half of the global cancer burden and the leukemia comprises of 3% of all malignancies with an incidence of 300,500 per year. The patterns of subtypes of leukemias vary in different populations and geographic areas. Here, we analyze both the hematological and non-hematological malignancies in the Western region of Nepal. This is a retrospective study done from Janauary 2000 to June 2011. All cases of bone marrow malignancies including leukemias, multiple myeloma and infiltration/metastasis to bone marrow were included in the study. Morphological subtypes {FAB (French, American and British) classification}, gender, age at diagnosis and peripheral blood counts of all the cases were analyzed. A total of 155 cases of hematological and non-hematological malignancies were reported during this period. Age ranged from 1 year to 82 years. Among 123 leukemia cases, acute myeloid leukemia (AML) was the commonest (80 cases) followed by chronic myeloid leukemia (CML) (20 cases), acute lymphoid leukemia (ALL) (16 cases) and chronic lymphoid leukemia (CLL) (7 cases). The commonest childhood leukemia below 15 years was AML followed by ALL. The commonest subtypes of AML and ALL were M2 and L2 respectively. CML cases in our study were less common and females were more affected than other parts of the country. There were 23 cases of multiple myeloma and 9 cases of infiltration/metastasis to marrow. Males were more affected by multiple myeloma in our study. Our data reflects bone marrow malignancies in the Western region of Nepal which is more comparable to the Central than Eastern region of Nepal.

Study of fine needle aspiration cytology of breast lumps and its histopathological correlation in Pokhara Valley.

Breast carcinoma is the leading cause of cancer death in women. Most of the time breast carcinoma presents as breast lump. Fine Needle Aspiration Cytology (FNAC) is a convenient and rapid preoperative diagnostic procedure. This is a prospective and correlative study done in department of Pathology of Manipal Teaching Hospital, Pokhara from December 2003 to December 2005. FNAC was performed in patients presenting with breast lump and its findings were correlated with histopathological findings. Out of total 343 cases of breast FNAC, 73 cases had histopathology correlation. Age group of the patient ranged from 17 to 84 years. Breast lumps were most commonly seen in age group 21 to 30 years which comprises of benign lesions. Maximum number of malignancy (26%) was seen in age group 41 to 50 years. Out of 73 cases, malignancy was seen in 15 cases (20.5%). The most common carcinoma was Invasive Ductal Carcinoma (IDC) with 46.7% cases. With correlation of FNAC and histopathology, the sensitivity and specificity of both benign and malignant lesions were high. In malignant lesions, the sensitivity and specificity were 93.3% and 100% respectively. FNAC is a safe diagnostic procedure in the preoperative diagnosis of breast lumps in our setting. It gives the accurate result with proper technique and interpretation.

Castleman's disease - hyaline vascular type - clinical, cytological and histological features with review of literature.

BACKGROUND AND AIMS: Castleman's disease (CD) is a rare disease of lymph node with two identified forms - the hyaline vascular (HV) type or angiofollicular type and plasma cell (PC) type. It usually presents as localized or systemic lymphadenopathy or even as extra nodal mass and may give rise to several differential diagnoses. Fine needle aspiration cytology (FNAC), as the initial investigation, may be misleading. Excision biopsy usually gives the diagnosis. This study analyzes the clinical, cytological and histological features of CD of HV type. MATERIALS AND METHODS: All the cases of CD reported between 2001 and March 2008 have been reviewed. Cytology and histopathology slides of all cases were reviewed by two reporting pathologists independently and the clinical records were analyzed. RESULT: We had five cases of CD all of which presented as cervical lymphadenopathy of 1 to 5 cms in young patients. Patients were mostly asymptomatic with all relevant investigations within normal limits. The cytology findings mostly showed a predominance of small lymphocytes with capillary fragments. On excision biopsy, all cases were diagnosed as CD of HV type. CONCLUSION: CD should be kept as a differential of lymphadenopathy, especially in asymptomatic and young patients.

Adenosquamous carcinoma of the lacrimal gland.

A 48-year-old man presented with proptosis of the left eye, which on excisional biopsy proved to be primary adenosquamous carcinoma of the lacrimal gland. The lesion was excised radically by a limited frontotemporo-orbitozygomatic approach and any further surgeries, such as exenteration, were avoided in view of the patient's wish to preserve the eye and vision. Long-term follow-up is planned to look for any metastasis or recurrence of the tumour. Primary adenosquamous carcinoma of the lacrimal gland is a very rare entity and, following a thorough literature review, only two cases of lacrimal gland adenosquamous carcinoma have been reported so far.

Tumour and tumour-like conditions of peripheral nerve origin: ten years' experience.

BACKGROUND: There are four major lesions which may arise in the peripheral nerve, namely neuroma, schwannoma, neurofibroma and malignant peripheral nerve sheath tumor. OBJECTIVE: In the present study we look into the spectrum of peripheral neural tumors including their age distribution site distribution and histopathology. MATERIALS AND METHODS: This is retrospective study conducted in the Dept of Pathology, Manipal Teaching Hospital, Pokhara. All histopathologically diagnosed cases of primary lesions of peripheral nerve during the period Jan 2000 to Nov 2009 were reviewed and the data were analysed. RESULT: A total of 114 cases of peripheral neural lesions were reported in the same period. Total number of nonmalignant cases was 106 (93%) while malignant cases were 8 (7%). Among the nonmalignant cases neurofibroma was the commonest (51 cases, 45% of all) closely followed by schwannoma (39 cases, 34% of all). Among the neurofibroma cases only 2 cases had multiple neurofibromatosis while others were solitary. Among the schwannoma cases 4 were diagnosed as ancient schwannoma with presence of bizarre cells with hyperchromatic nuclei. The commonest site involved for both schwannoma and neurofibroma was scalp-face-neck followed by back. The age range for schwannoma was 16 to 75 years whereas the same for the neurofibroma cases was 2 to 82 years. MPNST cases were seen in the age range of 40 to 72 with 3 cases in upper extremity, 3 in lower extremity and 1 each in lip and cheek. 1 case was diagnosed as cellular neurofibroma with atypia in tongue and was confirmed by S100. CONCLUSION: The majority of the tumor are benign and the commonest benign tumor was neurofibroma of sporadic type, closely followed by schwannoma. In our study the commonest site was scalp-face-neck unlike other studies. This may be due to more compliance of the patients due to cosmetic reason.

Bone marrow culture vs. blood culture in FUO.

INTRODUCTION: Bone marrow culture is considered superior to blood culture in evaluation of FUO. The aim of this study was to compare the usefulness of these two cultures. METHODS: A one year prospective cross sectional study was conducted to find out the usefulness of bone marrow culture and blood culture in the diagnosis of FUO. Marrow aspirates in each case were sent for bacterial, myocbacterial and fungal culture. Simultaneously venous blood was sent for bacterial culture. The results of BMCs and BCs were compared. RESULTS: Total 57 cases of FUO were included in the study. Male female ratio was 1.22:1. Age range was five to 83 years (median 30). Duration of fever was 21 to 365 days. Bacterial growth was seen in nine cases (15.78%) of BMCs and in three cases (5.26%) of corresponding BCs. Fungal or myocbacterial growth was not seen. Salmonella typhi was the commonest organism isolated in BMCs (three cases) followed by Staphylococcus aureus (two cases), Escherichia coli, Non fermenting Gram negative bacilli, Enterococcus species and Salmonella paratyphi-A (one case each). Two cases of Salmonella typhi and one case of Salmonella paratyphi-A were isolated in BCs. CONCLUSION: BMCs are more useful than BCs in evaluation of patients with FUO, especially in cases of salmonella infection and are particularly important when the patient has already taken antibiotics. In immuno-competent patients presenting with FUO, BMCs for mycobacteria or fungi is unlikely to yield any growth.

Reye syndrome: a case report with review of literature.

Here we describe a case of Reye syndrome diagnosed at postmortem liver biopsy of a three-year old girl who presented with vomiting, low grade fever for three days and loss of consciousness for 18 hours. Clinically, the differential diagnoses were meningitis, encephalitis and septicemia. No history of past illness or any drug ingestion including aspirin were present. Laboratory investigations indicated a diagnosis of Reye syndrome. The child was given supportive treatment but died after two days of admission and postmortem needle-biopsy of the liver showed microvesicular steatosis consistent with Reye syndrome.

Chromoblastomycosis in Nepal: a study of 13 cases.

BACKGROUND: Chromoblastomycosis is a chronic fungal infection caused by several pigmented fungi commonly seen in tropical and subtropical climates. AIM: To evaluate the epidemiologic, clinical and pathological characteristics of chromoblastomycosis in our patients. METHODS: This retrospective and prospective study was conducted at the Manipal Teaching Hospital, Pokhara, Nepal. Clinical features and histopathology of all the cases diagnosed as chromoblastomycosis during the last eight years were studied. RESULTS: A total of 13 cases of chromoblastomycosis were diagnosed during the period of 1999-2006. The disease was seen predominantly in middle-aged male farmers and those from rural areas. The lesions commonly involved the lower extremity and were single or multiple in number. They clinically presented as verrucous or nodular growths. Out of these 13 cases, three were diagnosed clinically as squamous cell carcinoma and one as psoriasis. The histopathological features included sclerotic bodies in 12 cases (92%), microabscess formation in 10 cases (76.9%), pseudoepitheliomatous hyperplasia in nine cases (69.2%) and granuloma in eight cases (61.5%). CONCLUSION: Farming is the commonest occupation in patients with chromoblastomycosis. Early histological diagnosis helps in effective management of the condition.

Nasal glioma: a case report.

Here we describe a rare case of nasal glioma in a 14 year old boy who presented with history of right nasal obstruction and nasal discharge since 6 months. On examination a pale mass was seen filling the right nasal cavity and extending down in the oropharynx. Clinical impression was antrochoanal polyp. Excision of the mass was carried out and sent for histopathology which showed presence of glial tissue and the diagnosis was made as nasal glioma.