Vaginal clear cell adenocarcinoma with associated Müllerian duct anomalies, renal agenesis and situs inversus: report of a case with no known in-utero exposure with diethyl stilboestrol.

A 27 year old female presented with two months history of continuous vaginal bleeding. On evaluation, she was diagnosed as a case of vaginal clear cell adenocarcinoma (VCCA) along with associated Müllerian duct anomalies including didelphys uterus with double vagina; left sided renal agenesis and situs inversus. Although VCCA is strongly linked to in-utero exposure with Diethyl stillboestrol (DES), this patient had no known history regarding such exposure. Also, only a few cases have been described in the literature in which this tumour was seen in association with Müllerian duct anomalies and renal agenesis; in the absence of known DES exposure.

Primary cutaneous B cell lymphoma--leg type (NEW EORTC--WHO classification), with nasal sinuses involvement.

Primary Cutaneous lymphomas of B cell origin are rare, there remains a controversy in truly classifying these lymphomas and an updated EORTC classification divides them on the basis of their distinct histopthological grounds rather than on the basis of their anatomic location as in WHO classification, while the new WHO- EORTC joint classification maintains some characteristics of both systems, We report an elderly gentleman who primarily had a typical Leg dominant Cutaneous lymphoma of B cell origin uniquely with involvement of nasal Sinusues, bearing the Immunohistochemical staining features of "Cutaneous lymphoma - Leg Type" befitting the new joint WHO-EORTC classification of Cutaneous B cell Lymphoma.

Clear cell papillary cystadenoma of epididymis, a mimic of metastatic renal cell carcinoma.

We discuss a case of 25 year old male who was evaluated for primary infertility following marriage. He had no previous history of urogenital complaints or abnormalities. In fact, his past medical history was unremarkable. On examination epididymal masses were found. Semen analysis showed azoospermia. Right epididymal mass was surgically excised. Histopathology showed an epididymal neoplasm composed of simple and complex papillary processes lining and filling the ducts. The tumor cells were clear and positive for cytoplasmic glycogen. Immunohistochemistry was also supportive. Diagnosis of clear cell papillary cystedenoma was made based on histopathological and immunohistochemical features.

Post-transplant T-cell type lymphoproliferative disorder.

Post-Transplant Lymphoproliferative Disorder (PTLD) is a lymphoma, which develops as a result of immunosuppression in a recipient of a solid organ or bone marrow allograft. Majority are associated with Ebstein-Barr Virus (EBV) infection, are mostly B-cell type and less often T-cell type. We report a case of T-cell PTLD, occurring in a renal transplant recipient.

Thymoma : a clinicopathologic association of world health organization histologic subtype and invasive behaviour.

OBJECTIVE: To re-classify thymic epithelial neoplasms reported at Aga Khan University Hospital during the past seven years according to the revised WHO classification, to assess the ease of application and determine association between WHO histological subtype and invasive behaviour. DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: The study was carried out in the section of Histopathology, Department of Pathology and Microbiology, Aga Khan University Hospital, Karachi, from January 2000 to October 2006. MATERIAL AND METHODS: All cases of thymic epithelial neoplasms reported in the past seven years were retrieved using SNOMED coding system. Small biopsies where the tissue was insufficient for definite classification were not included. All cases were reviewed and reclassified according to WHO classification into types A, AB, B1, B2 and B3. Capsular invasion as well as extension into neighboring structures such as perithymic fat, pleura, pericardium, lung etc. was noted on morphology. RESULTS: A total of 62 cases were diagnosed as Thymic Epithelial Tumors (TET). Out of these, there were 5 type A (6 %), 17 type AB (21%), 7 type B1 (8.6%), 26 type B2 (32%) and 7 type B3 (8.6%) thymomas. Age range was from 22-78 years with a median age of 46 years. Male to female ratio was 6:1. History of associated myasthenia gravis was present in 21% of cases. A significant association was observed between WHO histologic subtype and invasive behaviour where types A, AB and B1 have lesser number of invasive cases as compared to non-invasive, whereas in types B2 and B3, more cases have shown invasion as compared to non-invasive cases (c2 = 14.093, df =1, p-value < 0.001 ). CONCLUSION: The WHO classification is simple and easy to apply and has significant association with aggressive behavior. To some extent, it reflects the clinical behaviour of thymomas along with stage and status of resection. However, morphologically benign looking thymomas can behave aggressively. Hence, tumour stage, extent of resection and histology should be combined to predict the clinical behaviour of thymomas.

Gastric carcinoma: typing, staging, lymph node and resection margin status on gastrectomy specimens.

OBJECTIVE: To determine the extent and stage of gastric carcinoma on gastrectomy specimens in terms of type, grade, location, depth of invasion, resection margins and lymph node status. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Section of Histopathology, Department of Pathology, Aga Khan University, Karachi during 2005 and 2006. MATERIALS AND METHODS: A 100 consecutive gastrectomy specimens received during the study period, in which resection was done for gastric carcinoma were included. Exclusion criteria were gastrectomy performed for gastrointestinal stromal tumor (GIST) and gastric lymphoma. Specimens were examined according to standard techniques. The reporting incorporated all relevant parameters including gross examination, tumor location, type and grade, depth of invasion, surgical resection margins and lymph node status. Staging was done according to the TNM staging system. Restults: Fifty three (53%) tumors were located in the distal part of the stomach and 35 (35%) in the proximal part. In 12 (12%) cases, tumor was diffusely involving the entire stomach. Histologically, the tumors were signet ring type in 63 (63%) cases and intestinal type in 37 (37%). At least one peripheral resection margin was positive in 29 (29%) cases. Sixty six (66%) cases were T3, 30 (30%) were T2, 3 (3%) were T1 and one case (1%) was T4. Twenty four cases (24%) were N0, 35 (35%) were N1, 27 (27%) were N2 and 14 (14%) were N3. CONCLUSION: Gastric cancer was at very advanced stage in these patients with full thickness involvement of the gastric wall and positive lymph nodes. It is thus essential to detect and surgically treat these cancers in an early stage. Better surgical techniques are required to ensure negative peripheral resection margins and removal of adequate number of lymph nodes, so that proper staging can be performed.

Frequency of primary solid malignant neoplasms in different age groups as seen in our practice.

BACKGROUND: To determine in a large series of surgical biopsies, the frequency of various histologic types of primary solid malignant neoplasms in males and females in different age groups. METHODS: A retrospective study of 20,000 consecutive surgical biopsies reposted in the section of histopathology, AKU in 2004. RESULTS: Malignant neoplasms are commonest in the fifth and sixth decades of life. The commonest malignant neoplasms in the first decade were Hodgkin's lymphoma and Wilm's tumor in males and females respectively. In the second decade, osteosarcoma in males and Ewing's sarcoma / PNET in females. In the third decade, colorectal adenocarcinoma in males and infiltrating. Ductal carcinoma of breast in females. In the fourth decade, squamous cell carcinoma of oral cavity in males and infiltrating ductal carcinoma of breast in females. In the fifth decade squamous cell carcinoma of oral cavity in males and infiltrating ductal carcinoma of breast in females. In sixth decade, squamous cell carcinoma of oral cavity in males and infiltrating ductal carcinoma of breast in females and in the seventh decade, prostatic adenocarcinoma in males and infiltrating ductal carcinoma of the breast in females. Above age of 70 years, the commonest malignant were again prostatic adenocarcinoma in males and infiltrating ductal carcinoma of breast in females. CONCLUSION: Malignant neoplasms are commonest in the fourth, fifth and sixth decades of life.

Frequency of primary solid malignant neoplasms in both sexes, as seen in our practice.

BACKGROUND: To determine the frequency of various histologic types of primary solid malignant neoplasms in males and females, in our practice, in a large series of surgical biopsies. METHODS: A retrospective study of 20,000 consecutive surgical biopsies in the section of Histopathology, Aga Khan University Hospital (AKU), Karachi, in 2004. RESULTS: Squamous cell carcinoma of oral cavity was the commonest malignant neoplasm in males followed by diffuse Large B cell, Non-Hodgkin's lymphoma and Prostatic adenocarcinoma. In females, infiltrating Ductal carcinoma of the breast was overwhelmingly the commonest malignant neoplasm followed by Squamous cell carcinoma of the oral cavity and esophagus. CONCLUSION: Out of 20,000 biopsies, there were 4616 (23.08%) malignant neoplsms. Carcinoma of oral cavity is very common in our population in both sexes.

Subependymal giant cell astrocytoma.

Subependymal giant cell astrocytomas (SEGAs) are slowly growing tumours corresponding to WHO grade I. They are intraventricular and usually occur in the setting of tuberous sclerosis complex. They often result in obstructive hydrocephalus. Treatment is usually restricted to surgical resection, recurrences are rare and long term prognosis is excellent. We present a series of three cases.

Angiokeratoma of tongue: a series of 14 cases.

Angiokeratomas (AC) are vascular lesions which are defined histologically as one or more dilated blood vessels lying directly subepidermal and showing an epidermal proliferative reaction with ectatic capillaries in the papillary dermis. Only three other cases of isolated mucosal angiokeratoma have been reported in the indexed literature. We reviewed all cases of angiokeratoma located on the tongue, diagnosed in our department during a study period of 10 years (1995-2005). Histologically all 14 cases showed dilated and congested blood vessels in the upper papillary dermis. They lack deep dermal involvement. Hyperkeratosis and acanthosis were also seen in most of the cases. No clinical data was available to assess systemic disease. A higher incidence of of AC in tongue is seen in our study.

Is fascin really a useful marker in distinguishing between classical Hodgkin's lymphoma and various types of non-Hodgkin's lymphomas in difficult cases?

BACKGROUND: Fascin is an actin cross-linking protein, which is part of the cytoskeleton and involved in cell motility in several cell types. The differentiation of epithelial cells is greatly influenced by cell-matrix and cell-cell interactions, which play an important role in the normal organisation and stabilisation of epithelial cells and maintain the cells in a non-migratory state. The malignant conversion of epithelial cells results from a phenotypic switch to a migratory state, which allows tumour invasion beyond the basement membrane, as well as metastasis. Such a switch requires complex rearrangements of the actin cytoskeleton and is governed by multiple actin-binding proteins including fascin. In non-neoplastic lymphoid tissue, fascin expression is highly selective and is predominantly localised in dendritic cells, while lymphocytes, plasma cells, etc are uniformly non-reactive. However, all or nearly all Reed-Sternberg cells and their variants in all types of Hodgkin's lymphoma express fascin and are strongly immunoreactive for fascin. CONCLUSION: Lymphomas (Hodgkin's and non-Hodgkin's) are among the commonest malignancies seen in our practice. The Section of Histopathology at the Aga Khan University, Karachi is the largest centre for histopathology in Pakistan, a densely populated country with an estimated population of 170 million. The authors wanted to test the utility of Fascin in distinguishing between Hodgkin's lymphoma and morphologically closely related forms of non-Hodgkin's lymphoma such as diffuse large B cell non-Hodgkin's lymphoma and anaplastic large cell lymphoma in difficult cases. If found useful, this antibody could help us in reaching a correct diagnosis in difficult cases and allow appropriate patient management.

Ultrastaging of sentinel lymph nodes (SLNs) vs. non-SLNs in colorectal cancer--do we need both?

BACKGROUND: The aim of this study to analyze whether ultrastaging of initially negative nonsentinel lymph nodes (non-SLNs) would increase nodal positivity in colon cancer and rectal cancer. METHODS: After SLN mapping (SLNM), SLNs were ultrastaged by 4 hematoxylin and eosin and 1 immunohistochemistry sections. A blinded pathologist reexamined initially negative non-SLNs by 3 additional hematoxylin and eosin and 1 immunohistochemistry sections. RESULTS: In 156 colon cancer and 44 rectal cancer patients, 2,755 nodes were identified (494 SLNs and 2,261 non-SLNs). Metastases were detected in 20.9% of SLNs and 8.6% of non-SLNs (P<.0001). After ultrastaging non-SLNs, only .58% became positive for metastases in 12 patients. Of these, 10 already had positive lymph nodes, hence no change of staging occurred. Ultrastaging upstaged only 2 of 200 patients (1%). CONCLUSIONS: The chance of finding additional metastases by ultrastaging of all non-SLNs is extremely low (<1%) and of little benefit.