Primary small cell neuroendocrine cancer of adrenal gland: a rare extrapulmonary small cell cancer.

Small cell cancer is an aggressive neoplasm of neuroendocrine origin that is most commonly found in the lungs. However, up to 5% of cases can be extrapulmonary. These have been reported to be located in the gastrointestinal and genitourinary systems and rarely in other locations. Only five prior cases of small cell cancer have been reported where the primary lesion is at the adrenal gland. Here we present the case of a female patient in her mid-50s who presented with right upper quadrant pain and was diagnosed with metastatic small cell carcinoma of the adrenal gland. The patient received palliative chemotherapy for her metastatic cancer and was ultimately admitted to hospice after progression of her disease. This case and the accompanying literature review highlight a rare manifestation of extrapulmonary small cell cancer.

Frequency Of Double Expressor Lymphoma In A Tertiary Care Hospital.

BACKGROUND: Accounting for 30% cases of all lymphoid neoplasms, Diffuse large B cell lymphoma (DLBCL) is the commonest lymphoma worldwide. It occurs over a wide age range and has diverse morphology, immunophenotype and clinical outcome. Objectives of the study were to determine the frequency of Double expressor lymphoma (DEL) in a tertiary care hospital. METHODS: This descriptive cross-sectional study was carried out in the Department of Histopathology, Rehman Medical Institute Peshawar from June 1st to December 1st, 2018. A total of 88 newly diagnosed cases of diffuse large B-cell lymphoma (DLBCL); diagnosed on incisional or excisional biopsies were included in the study by non-probability consecutive sampling. Statistical analysis was carried out using SPSS-23. Quantitative variables were calculated as mean±SD. Qualitative variables were computed as frequency and percentages. Post stratification chi-square test was applied keeping p value equal or less than 0.05 as significance. RESULTS: In our 88 cases of DLBCL, 56 (63.6%) were males and 32 (36.4%) were females. Age of patients ranged from 15yrs to 84yrs. Mean age was 50.8±15.2SD. Activated B-cell like (ABC) subtype of DLBCL constitute 51 cases (58%) while 37 cases (42%) were of germinal centre B-cell like (GCB) subtype. Nineteen cases (21.6%) were of DEL. Cervical node was the commonest site of involvement (n=17, 19.3%) followed by stomach (n=10, 11.4%) and tonsil (n=6, 6.8%). Out of 19 cases of DEL, 17 cases (89.5%) were of ABC type. DEL was found to have significant correlation with ABC subtype of DLBCL (p=0.002). DEL had no correlation with gender (p=0.6), age (p=0.27), Mib-1 (p=0.36) and tumour site (p=0.42). CONCLUSIONS: The frequency of DEL in our study was 21.6% which is comparable to other studies who used similar cut-offs for c-Myc and BCL2 and similar criteria of inclusion as in our study. Significant association was found between DEL and ABC subtype of DLBCL.

Frequency of Human Epidermal Growth Factor Receptor 2 (Her2/Neu) Expression in Gastric Adenocarcinoma in Rehman Medical Institute Peshawar.

OBJECTIVE: To investigate the frequency of Human Epidermal Growth Factor Receptor 2 over expression in gastric adenocarcinoma by immunohistochemistry and to find the association of its expression with clinicopathological parameters. . METHODS: The descriptive cross-sectional study was conducted at Rehman Medical Institute, Peshawar, Pakistan, from January to December 2016, and comprised consecutive formalin-fixed and paraffin-embedded samples of gastric adenocarcinoma. The cases were scored for Human Epidermal Growth Factor Receptor 2 expression according to criteria cited in Trastuzumab for Gastric Cancer trial. Correlation of the expression with different clinicopathological parameters was determined. SPSS 23 was used for data analysis. RESULTS: Of the 55 cases, 49(89%) were biopsies and 6(11%) were gastrectomies. Among the patients whose samples were tested, 41(74.5%) were male. The overall mean age was 59.16}12.58 years (range: 38-95 years). Human Epidermal Growth Factor Receptor 2 overexpression (3+) was present in 19(34.5%) cases.Out of 21(38.2%) cases of moderately differentiated adenocarcinoma, 10(47.6%) showed overexpression. It was commonest in tumours of the fundus area 7(31.6%). No association of the expression was found with tumour's histological grade and location, or with patient's gender and age (p>0.05 each).. CONCLUSIONS: More than one-third of the sample had overexpression of Human Epidermal Growth Factor Receptor 2.

Plasma Cell Leukaemia: A Rare Yet Aggressive Plasma Cell Dyscrasia With A Very Poor Response To Conventional Therapy.

Plasma cell leukaemia (PCL) is a very rare plasma cell dyscrasia with a significant number of monoclonal plasma cells in the peripheral blood. It is diagnosed by the presence of ≥ 2x109 /L plasma cells in the blood or by plasma cells making up ≥20% of the leukocyte count. It can arise from a leukemic transformation of multiple myeloma, or more commonly it can be primary. Regardless of its origin, it carries a very dire prognosis. It responds very poorly to the traditional chemotherapy regimens used for multiple myeloma. We present the case of a 50 years old female who presented to our hospital with a complicated UTI and severe generalized body aches. She was diagnosed as a case of plasma cell leukaemia and was treated with cyclophosphamide and dexamethasone, however she failed to go into remission. Her condition deteriorated and she ultimately passed away 1.5 months after diagnosis. The recommended treatment for PCL is aggressive combination chemotherapy followed by stem cell transplantation. However, there is no consensus regarding the treatment of plasma cell leukaemia, and treatment should be individualized based on the patient profile. Once diagnosed, the prognosis is poor.

Frequency of human papilloma virus in patients with oesophageal squamous cell carcinoma in Khyber Pakhtunkhwa.

To determine the frequency of human papilloma virus (HPV) in patients with oesophageal squamous cell carcinoma. A descriptive, cross-sectional method was adopted and the study was conducted in the histopathology department of Rehman Medical Institute, Peshawar, Pakistan, from January to June 2017, and included patients with Oesophageal squamous cell carcinoma. Non-probability consecutive sampling technique was used. SPSS v.22 was used for data analysis. Out of 121 patients, 67(55.37%) were male and 54(44.62%) were female. The overall mean age was 50±1.72 years. Most of the patients were in the age group of over 50 years and only 12% were in the age group below 40 years. In our study patients with oesophageal squamous cell carcinma, human papillomavirus was found in 3%.

Terminal deoxynucleotidyl transferase (TdT)-negative T-cell lymphoblastic lymphoma with loss of the T-cell lineage-specific marker CD3 at relapse: a rare entity with an aggressive outcome.

Terminal deoxynucleotidyl transferase (TdT)-negative T-cell lymphoblastic lymphoma is a variant of T-cell lymphoblastic lymphoma/T-cell lymphoblastic leukaemia. TdT is a marker of immaturity expressed in 90%-95% cases of lymphoblastic lymphoma and useful in differentiating it from other mature lymphomas/leukaemias. It has been associated with poorer response to chemotherapy and a more aggressive outcome. Here we present a case of TdT-negative T-cell lymphoblastic lymphoma in a 28-year-old man who presented with superior vena cava syndrome. The patient was treated with hyper-cyclophosphamide,vincristine, Adriamycin, dexamethasone (CVAD), however unfortunately suffered a relapse 1 year later. A unique feature of our case was that on relapse, the patient lost expression of the T-cell lineage-specific marker CD3, which has previously not been reported in association with TdT-negative T-cell lymphoblastic lymphoma. The patient failed to respond to chemotherapy on his relapse and died.

Follicular dendritic cell sarcoma presenting as colonic intussusception.

Follicular dendritic cell sarcoma (FDCS) is a rare intermediate grade sarcoma involving a variety of nodal and extra nodal sites. It has two histological subtypes, conventional and inflammatory pseudotumour like variant. We report this interesting case of FDCS presenting colonic intussusception at Shifa International Hospital, Islamabad, Pakistan. Conventional FDCS presenting as a colocolic intussusception is an unusual presentation, and to our knowledge, has never been reported previously. It has wide morphological spectrum on light microscopy and has characteristic immune-reactivity for dendritic cell markers (CD21, CD23, and CD35). Surgical excision is required in all cases while role of adjuvant chemotherapy and radiotherapy is not clearly demonstrated in literature.

Incidental Squamous Cell Carcinoma Of The Renal Pelvis In A Non Functioning Kidney That Was Missed On Two Non-Contrast Ct-Scans.

Although the second most common malignancy after urothelial carcinoma, squamous cell carcinoma (SCC) of the renal pelvis is a rare entity. It has strong association with nephrolithiasis thus emphasizing prompt treatment of renal calculi. Because of rarity and nonspecific clinical and radiological findings, it mostly presents at pT3 or higher stage. We report SCC of renal pelvis that was missed two times on non-contrast CT scans and was diagnosed incidentally in a nephrectomy specimen. Its prognosis is similar to that of urothelial carcinoma of comparable stage. Owing to its rarity, no standard treatment guidelines are available; however radical nephrectomy with lymph node dissection is the initial treatment which can be curative in an early stage disease. Adjuvant chemotherapy and radiotherapy are usually ineffective.

Sex cord stromal tumours of the ovary, experience at Shifa International Hospital Islamabad.

This descriptive study was carried out at Pathology Department, Shifa International Hospital from 2007 to 2016; all sex cord stromal tumours diagnosed during this time period were included. Epithelial, germ cell and metastatic tumours were excluded from the study. A total of 1254 Ovarian tumours were brought to Shifa of which47 (4%) were labeled as sex cord stromal tumours. Of these 36( 76 %)were granulosa cell tumour (adult33, juvenile3), 7 were labeled as sertoli leydig cell tumours (15%), 3 as thecoma/ fibroma group (7%)and only one case was labeled as microcystic stromal tumour of the ovary (2%). Overall age range for sex cord stromal tumours was 42 (12-71). Immunohistochemistry was done in 41 out of 47 cases. Sex cord stromal tumours of the ovary are rare tumours comprising 4% of the total. Adult Granulosa cell tumour is the commonest tumour seen in our study.

Primary cardiac leiomyosarcoma presenting as haemoptysis in a 22-year-old patient: an unusual presentation of a rare condition.

Primary cardiac sarcomas are rare malignant tumours and among them, leiomyosarcoma is extremely rare. They are especially rare in a young age group. We present a case of a 22-year-old male patient with primary leiomyosarcoma of the left atrium involving the pulmonary veins who presented with haemoptysis and shortness of breath. He underwent surgical excision of the tumour along with mitral valve replacement followed by adjuvant chemotherapy. No recurrence was reported on his second 6 monthly follow-ups.

Recurrent verrucous carcinoma of the urinary bladder after transurethral resection followed by intravesical mitomycin, and a review of the literature.

Verrucous carcinoma (VC) is a locally invasive, non-metastasising well differentiated variant of squamous cell carcinoma. It is very rare in the urinary bladder and mostly associated with schistosomiasis. It has a characteristic multilobular appearance with small surface projections on contrast cystogram. Owing to its bland cytology, it is a challenging diagnosis. We report a case of VC of the bladder-unrelated to schistosomiasis and involving the prostate-that recurred after transurethral resection of the tumour and prostate followed by six doses of intravesical mitomycin. To the best of our knowledge, no such case has been reported from Pakistan.