Clear cell carcinoma of the breast with immunohistochemical evidence of divergent differentiation.

Primary clear cell carcinoma of the breast is a rare tumor. The clear cell morphology of the neoplastic population in these tumors has been ascribed to the presence of intracellular lipid, mucin or glycogen, or to myoepithelial, apocrine, or neuroendocrine differentiation. However, a clear cell neoplasm exhibiting evidence of a range of differentiation has not been previously reported. We describe a case of a glycogen-rich primary clear cell breast carcinoma occurring in a 59-year-old woman that showed positivity for apocrine and neuroendocrine markers, as well as possible myoepithelial differentiation. The tumor was a 4-cm mass composed predominantly of periodic acid-Schiff-positive clear cells arranged in a solid, infiltrative pattern. Immunohistochemical staining of the tumor cells was variably positive for cytokeratin, progesterone receptors, gross cystic disease fluid protein-15, neuron specific enolase, chromogranin, and S-100 protein and negative for estrogen receptors, smooth muscle actin, CD31, and CD34. The patient refused any form of further investigation or treatment, but shows no evidence of recurrence or metastatic disease after 18 months of follow-up.

Ovarian hyperstimulation syndrome associated with clomiphene Citrate

Ovarian hyperstimulation is a recognized complication of ovulation induction with gonadotrophins. The syndrome is becoming more common as the number of women undergoing in-vitro fertilization increases. It is rarely seen in conjunction with clomiphene citrate usage. This case report is of moderate to severe ovarian hyperstimulation in a patient who was treated with clomiphene citrate because of infertility secondary to anovulation. She presented with amenorrhoea for five weeks, lower abdominal pain and a positive urinary human chronic gonadotrophin (hCG) test. Pelvic ultrasonography was suggestive of a possible ectopic pregnancy with a different diagnosis of a ruptured ovarian cyst. Diagnostic laparoscopy was done followed by laparotomy. Oophorectomy was performed because the ovary was thought to be complex with solid areas. However, conservative management with advance of laparotomy is the recommendation in confirmed cases of ovarian hyperstimulation but this requires a high level of suspicion in patients who have ovulation induction. (AU)

Male breast cancer - an analysis of four cases and review of the literature

We report the cliniopathological features and outcome of four cases of carcinoma of the male breast diagnosed at the University Hospital of the West Indies between 1988 and 1998. Male breast cancer is most commonly seen in men over age sixty years, and it exhibits the same prognosis, stage for stage, as the disease in females. However, some researchers report overall poorer survival in men, and explain this on the basis of advanced disease at presentation. Other researchers maintain that the majority of breast cancers in males present at early stages, and exhibits survival similar to that in females. The published literature concerning this uncommon malignancy is reviewed, and the data are compared with the features of our cases.(Au)

Clear cell sarcoma of soft parts. A case report and review of the literature

The case of a 25-year old woman with recurring and metastasizing clear cell sarcoma of parts (CCS) is presented. The clinical setting and pathological apperance were in agreement with the original cases described. This report serves to highlight the clinico-pathological features of this rare and aggressive malignancy with specific reference to prognostic markers and treatment. (AU)

Medical students' evaluation of their patholgy clerkship experience

As part of a recent trend among faculties in educational institutions to review and upgrade their courses and teaching methods, the Department of Pathology introduced a questionnaire for assesment of the pathology clerkship experience by the medical students. This was to be completed at the end of their rotation. An elevation of the results of an entire class indicates that, for the most part, the responses were positive with 82 per cent considering the experience to be of great benefit and 17 per cent of reasonable benefit. The responses were generally more favorable to Anatomical Pathology and Haematology than to Chemical Pathology. The study identified areas where modifications in teaching could be considered, and ways to improve the questionnaire, making it more useful for future assesments. (AU)

The ectopic ACTH Syndrome and superior vena cava obstruction due to a malignant carcinoid tumour of the thymus

We report the case of a 28-year-old African Caribbean woman with Cushing's syndrome and superior vena cava obstruction secondary to an ACTH-secreting carcinoid tumour of the thymus. The case highlights the problems which may be encountered in performing the 2-day high dose dexamethasone suppression test but clinicians are reminded that this test or any other dynamic test is absolutely essential for elucidating the cause of ACTH-dependent Cushing's Syndrome (AU)

Malignant lymphoma of gastric mucosa-associated lymphoid tissue in Jamaica

The recently appreciated concept of Mucosa-Associated Lymphoid Tissue (MALT) and the characteristics of the lymphomas arising therein are discussed with reference to the clinical, histological and immunohistochemical features of the first four cases of gastric MALT lymphomas diagnosed at the University Hospital of the West Indies. These tumours are low-grade B-cell lymphoas, which may undergo high-grade transformation. They are aetiologically associated with Helicobacter pylori (H pylori) infection in the stomach and may be cured in the early stages with antibiotics. Our cases were diagnosed from gastrectomy specimens removed for suspected carcinoma. All were high-grade, and associated with H pylori; 3 cases presented as advanced disease. Documentation of the features of these lymphomas will increase awareness and earlier recognition (AU)

Angiomyofibroblastoma - a rare but distinct entity

A case of angiomyofibroblastoma of the vulva in a 23-year-old woman is presented. Clinical, histological and immunohistochemical features as well as the differential diagnosis and prognosis of this rare but distinct entity are discussed. Only about 65 cases are documented in the literature and to our knowledge this is the first case to be reported from the Caribbean(AU)

Differential patterns of serum biomarkers of immune activation in human T-cell lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis, and adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATL) and human T-cell lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) are associated with differing patterns of immune dysfunction. Biomarkers of immune activation may correlate with perturbations of immune function associated with these diseases. We conducted a pilot cross-sectionalstudy to assess four candidate biomarkers of immune activation, beta 2-microglobulin, neopterin, tryptophan, and kynurenine levels were assayed in storedsera from asymptomatic, human T-cell leukemia virus type I (HTL V-I) seronegative (HTLV-I-) and HTLV-I-seropositive (HTLV-I+) individuals, and ATL and HAM/TSP patients previously enrolled in seroepidemiological studies in Jamaica. Mean levels of beta 2-microglobulin, neopterin, and kynurenine were significantly elevated among ATL patients compared to the other study groups. Mean tryptophan levels were signigicantly lower among ATL and HAM/TSP patients than HTLV-I- and HTLV-I+ groups. No significant differences in biomarkers were found between HTLV-I- and HTLV-I+ groups. Among HAM/TSP patients, a significant association was found between elevated neopterin levels and symptoms of less than 4 years duration. In Cox proportional hazards regression modeling, neopterin and tryptophan were found to be independent predictors of survival among ATL patients. This study demonstrates a differential pattern of biomarkers of immune activation among ATL and HAM/TSP patients compared to HTLV-I- amd HTLV-I+ individuals. Neopterin and tryptophan may be useful clinical indicators of disease severity and prognosis among HAM/TSP and ATL patients. (AU)

Mammary hamartoma: an under-recognized breast legion

The clinical, histological and immunohistochemical features of the first three cases of mammary hamartoma diagnosed at the University of the West Indies are reported. These tumours are innocuous and under-recognized although the mammographic features are characteristic. With increased utilization of this test in breast cancer detection in our region, it is likely that more of these legions will be identified.

Mammary hamartoma: an under-recognized breast legion

The clinical, histological and immunohistochemical features of the first three cases of mammary hamartoma diagnosed at the University of the West Indies are reported. These tumours are innocuous and under-recognized although the mammographic features are characteristic. With increased utilization of this test in breast cancer detection in our region, it is likely that more of these legions will be identified. (AU)

Clinical and laboratory features of adult T-cell leukaemia lymphoma in Barbados

We describe the clinical and pathological features of 23 Afro-Caribbean patients with adult T-cell leukaemia/lymphoma admitted to the Queen Elizabeth Hospital, Barbados over a 5 year period. There were 9 males and 14 females, with a median age of 38 years (range 14-84). Twelve had acute leukaemia, 10 lymphoma (including 4 with solitary extra nodal lesions) and 1 smouldering subtype. Two patients had a past history of tropical spastic paraparesis/HTLV I associated myelopathy (TSP/HAM). The prognosis was poor, with only 3 complete responses to chemotherapy (CHOP) lasting from 9 to 36 months. We conclude that ATLL in Barbados is similar to the disease in other Caribbean islands and Japan, except that in Barbados the age of onset is over a decade younger than in Japan.(AU)

Clinical and laboratory features of adult T-cell leukemia lymphoma in Barbados

We describe the clinical and pathological features of 23 Afro-Caribbean patients with adult T-cell leukemia/lymphoma admitted to the Queen Elizabeth Hospital, Barbados over a 5 year period. There were 9 males and 14 females, with a median age of 38 years (range 14-84). Twelve had acute leukemia, 10 lymphoma (including 4 with solitary extra nodal lesions) and 1 smouldering subtype. Two patients had a past history of tropical spastic paraparesis/HTLV I associated myelopathy (TSM/HAM). The prognosis was poor, with only 3 complete responses to chemotherapy (CHOP) lasting from 9 to 36 months. We conclude that ATLL in Barbados is similar to the disease in the other Caribbean islands and Japan, except that in Barbados the age of onset is over a decade younger than in Japan.(AU)

Malignant triton tumour of the spine

A malignant triton tumour is a malignant schwannoma with rhabdomyoblastic differentiation, and is a very rare occurrence. We describe the case of a 33-year-old man with neurofibromatosis type 1 who presented with paraplegia. Pathological examination of an intradural, extramedullary tumour excised at lumbar laminectomy demonstrated a malignant schwannoma with rhabdomyoblastic differentiation. We believe this is the first documented case of a malignant triton tumour of the spine

Malignant triton tumour of the spine

A malignant triton tumour is a malignant schwannoma with rhabdomyoblastic differentiation, and is a very rare occurrence. We describe the case of a 33-year-old man with neurofibromatosis type 1 who presented with paraplegia. Pathological examination of an intradural, extramedullary tumour excised at lumbar laminectomy demonstrated a malignant schwannoma with rhabdomyoblastic differentiation. We believe this is the first documented case of a malignant triton tumour of the spine (AU)

Effect of human T-lymphotropic virus type I infection on non-Hodgkin's lymphoma incidence

We previously reported from a case-control analysis that T-cell non-Hodgkin's lymphoma (NHL) was strongly associated with human T-lymphomphotropic virus type I (HTLV-I) infection in Jamaica and Trinidad and that the relative risk for HTLV-I infection was very high in younger patients. Purpose: the objective of this study was to estimate the age-specific incidence rates of NHL among HTLV-I-infected and HTLV-I-uninfected adults in Jamaica and Trinidad. Methods: Population rates of HTLV-I infection were calculated from available census reports and serosurvey data. Incidence rates for NHL were calculated from all incident cases in Jamaica during 1984-1987 (n = 135) and from all incident cases in Trinidad during 1986-1990 (n = 117). Using biopsy material, we determined whether the immunophenotype or the tumor cells was T cell, B cell, or other. NHL incidence rates were computed according to HTLV-I status, age, sex, and tumor phenotype for each country separately and for both countries combined by weighting to the relative population size of each country. Results: The age-standardized NHL incidence rate (mean ñ SE) in Jamaica was 1.9 ñ 0.2 per 100,000 person-years (PY). In Trinidad, the rate was 2.9 ñ 0.4 per 100,000 PY. Overall, the incidence of NHL increased with age and was higher in males than in females. In the HTLV-I-infected population, the incidence of NHL was inversely related to age, and age-specific rates were higher in males than in females. The NHL incidence in those estimated to have acquired HTLV-I infection in childhood, however, showed no sex difference, and one in 1300 such carriers (95 percent confidence interval: one in 1100 to one in 1600) per annum were estimated to be at such risk. For T-cell NHL, as proxy for adult T-cell lymphoma/leukemia, incidence was highest in those patients infected with HTLV-I early in life (perinatally or via breast milk), with high, sustained risk from early adulthood in both sexes. Conclusions: While overall NHL incidence rates reveal that HTLV-I endemicity does not impose an exaggerated lymphoma burden on these populations, the risk for lymphoma among carriers who acquire infection early in life is dramatic and is consistent with the hypothesis that virus exposure early in life is most important for lymphomagenesis. Implications: Studies of HTLV-I carriers known to be infected in childhood may provide insight into markers intermediate in the lymphomagnetic process. Strategies to disrupt early-life transmission of HTLV-I, notably mother-infant transmission, may be critical in reducing the burden of lymphoreticular disease in these populations (AU)

Splenic cysts - a Jamaican experience

A total of eleven (11) cases of non-parasitic splenic cysts have been studied at the University Hospital of the West Indies by routine haematoxylin and eosine and immunohistochemcial staining, using epithelial markers (cytokeratin, low molecular weight keratin and epithelial membrane antigen). The majority (eight of eleven) were found to epithelial or true cysts. This is in contrast to findings previously reported in the literature, based on light microscopic examination, that the majority of splenic cysts are non-epithelial in origin. We therefore suggest that immunohistochemical studies should be used routinely to accurately define the nature of all splenic cysts.

Splenic cysts - a Jamaican experience

A total of eleven (11) cases of non-parasitic splenic cysts have been studied at the University Hospital of the West Indies by routine haematoxylin and eosine and immunohistochemical staining, using epithelial markers (cytokeratin, low molecular weight keratin and epithelial membrane antigen). The majority (eight of eleven) were found to epithelial or true cysts. This is in contrast to findings previously reported in the literature, based on light microscopic examination, that the majority of splenic cysts are non-epithelial in origin. We therefore suggest that immunohistochemical studies should be used routinely to accurately define the nature of all splenic cysts. (AU)

Splenic cysts: a Jamaican experience

Epithelial cysts of the spleen are common and comprises approximately 10 percent of all non-parasitic cystic lesions. They are more common in females and are usually found in children and young adults. They are considered to be congenital in origin but the actual pathogenetic mechanism is not known. However, it is said to be due to metaplasia of the celomic epithelium or heterotopic endodermal inclusions within the spleen. It is also believed to be due to an embryonic defect in the aggregation of the mesodermal island. These cysts are usually large, unilocular or multilocular with fibrous trabeculation of the wall which is lined at least in part by stratified squamous epithelium. They are filled with yellow and brownish hemorrhagic or clear straw colour fluid. They vary in size. This communication describes eight such cases, one of which was reported in 1961 by Gore and Harland, found in the files of Pathology Department between 1953 to 1991. The age ranges between 10 - 31 years with 62 percent of it being females. The size of the spleen varies between 230 gm to 2,300 gm. Immunohistochemical methods were used to demonstrate the epithelial nature of the lining of the cysts in some cases. Clinical presentation, pathological findings, pathogenesis and treatment are discussed. (AU)