Postmenopausal hyperandrogenism due to an ovarian sex cord-stromal tumour causing elevated dehydroepiandrosterone sulphate: a case report.

BACKGROUND: The source of excess androgen can be obscure in postmenopausal women with new-onset hyperandrogenism. If serum dehydroepiandrosterone sulphate (DHEAS) is raised, it is presumed to be of adrenal origin because DHEAS is exclusively produced from adrenal cortical cells. This reports an elderly female presenting with new-onset hyperandrogenism due to an ovarian sex cord-stromal tumour, associated with increased serum DHEAS levels. CASE DESCRIPTION: A 76-year-old female with long-standing diabetes and hypertension presented with hirsutism and male type alopecia for six months. She had menopause at 55 years of age. There was a pelvic mass on examination. Total testosterone was 6.106 ng/ml (0.124-0.357) and DHEAS was > 1000 µg/dL (35-430). Contrast-enhanced computed tomography of the abdomen and pelvis showed a heterogeneously enhancing complex mass measuring 11 × 8 cm in the left adnexal region. Adrenal glands were normal. She underwent total abdominal hysterectomy, bilateral salphingo-oophorectomy, and omentectomy. Both testosterone and DHEAS normalised following surgery. Histology revealed a sex cord-stromal tumour, likely a steroid cell tumour with malignant potential. Fluorodeoxyglucose-Positron emission tomography did not show any additional lesions. CONCLUSIONS: Due to the lack of sulfotransferase in ovarian tissue, markedly elevated DHEAS originating from an ovarian neoplasm is unusual. This phenomenon has not been described except in a patient with a steroid cell tumour causing Cushing syndrome and hyperandrogenism. The mechanism of this rare occurrence remains elusive. Knowledge of this unusual presentation would enable the clinicians to be cautious in localising the androgen source in women with hyperandrogenism.

Bladder cancer in women: a Sri Lankan study

Introduction: Bladder cancer (BC) is the ninth commonest cancer globally, and ranks 19th among women. The literature on BC in women is sparse, and the knowledge on the variations of tumour characteristics in South Asian female BC is limited. The objective of this study was to describe the clinicopathological characteristics of BC in women and compare them with male BC. Methods: A retrospective analysis of all female patients with histopathologically proven newly diagnosed primary BC from January 2007 to January 2017 was done at the Urology Unit, National Hospital of Sri Lanka, and the findings were compared with men with BC during the same period. Results: There were 314 patients with primary BC, of which 55(17.5%) were women, with a male:female ratio of 4.7:1. Only 70.9% of women had haematuria at presentation. Forty-four women (80%) had urothelial bladder cancers(UBC). The mean age of women with UBC was 67±SD13.2 years, and for non-urothelial bladder cancers(NUBC) was 62.5±SD 12.7 years. NUBC in women accounted for a disproportionately higher percentage in comparison to men (20% vs.5.4%, p<0.001). Of the UBCs, muscle invasive BC accounted for 45.5% (20/44) in women as opposed to 33.5% (82/245) in men. Women had a significantly higher proportion of solid tumours (40%vs.22.8%,p=0.008) compared to men. Conclusion: Primary BC in women has a significantly higher proportion of NUBC and muscle invasive UBC, which are more aggressive cancers, compared to men. Furthermore, the lack of haematuria in >25%, and higher incidental diagnosis, with inherently thinner bladder wall, makes primary BC in women more likely to present late, in a more advanced state.

Primary Signet-Ring Cell Adenocarcinoma of the Urinary Bladder Treated with Partial Cystectomy: A Case Report and Review of the Literature.

Primary signet-ring cell carcinoma is a variant of adenocarcinoma which is extremely rare, associated with poor prognosis and generally found to be resistant to chemotherapy and radiotherapy. We report a case of primary signet-ring cell carcinoma of the bladder which was successfully treated with partial cystectomy. A 71-year-old female with a history of type 2 diabetes, hypertension, and ischaemic heart disease presented with painless haematuria for 2 months' duration. The abdominal ultrasonography showed a localised polypoidal vesical growth arising from the bladder dome. Cystoscopy revealed an exophytic solid tumour in the anterior fundal wall. A deep transurethral resection of bladder tumour was done and histology revealed an adenocarcinoma composed of mucinous and signet-ring cell components. Later, considering the patient's age and the poor general condition, a partial cystectomy was done. Follow-up cystoscopy and ultrasonography were done at 12 months and there was no evidence of tumour recurrence and the patient is currently symptom-free. Partial cystectomy may be considered in patients with localised tumour without evidence of metastasis and poor general condition. Regular cystoscopies and ultrasound imaging are necessary for follow-up and early identification of recurrences.

Hypopigmented mycosis fungoides in type v skin: a report of 5 cases.

Five patients with type V skin were studied to describe the clinical manifestations, pathological features, and treatment response in hypopigmented mycosis fungoides (HMF). The mean age of patients was 22.4 years at diagnosis, with a mean of 36 months of diagnostic delay. Two were children aged 11 and 13 years. Skin patches were limited to sunlight-covered body areas. In tropical climate, exposure to natural sunlight possibly cured the lesions on sun-exposed areas at early stage of onset. HMF may frequently be misinterpreted as eczema, vitiligo, or progressive macular hypomelanosis clinically and histopathologically as seen in our case series.

Prognostic factors associated with local recurrence, metastases, and tumor-related death in patients with synovial sarcoma.

Prognostic factors associated with local recurrence, metastases, and tumor-related death in synovial sarcoma were studied in 51 patients in the Scottish Bone Tumor Registry from 1955 to 1999. In a multivariate analysis, the presence of poorly differentiated (PD) areas was the strongest prognostic factor associated with local recurrence (Hazard ratio [HR] = 11.3, 95% CI 2.3, 122.5, p = 0.033), metastases (HR = 16.9, 95% CI 2.3,122.5, p = 0.005), and tumor-related death (HR = 6.9, 95% CI 1.1,41.8, p = 0.036). Other significant independent risk factors included bone invasion (HR = 16.6, 95% CI 1.1, 252.5, p = 0.043) and necrosis (HR = 5.1, 95% CI 1.4, 18.99, p = 0.016) for metastases and bone invasion (HR = 17.6, 95% CI 1.2, 253.2, p = 0.035) for tumor-related death. Increasing percentages of PD areas and necrosis were associated with increasing hazard ratios for metastases and death. In the univariate analysis, PD areas, tumor size, and a mitotic count over 10/10 high-power fields were significantly associated with recurrence, whereas necrosis, vascular invasion, and age more than 25 years were additional risk factors for metastases and death. Local recurrence was significantly associated with increased risks for metastases (OR = 6.8, 95% CI 1.6, 28.7, p = 0.006), and death (all cases). Histologic features such as PD areas, necrosis, vascular invasion, and bone invasion should be considered when deciding about adjuvant therapy.

Chondrosarcoma of the bones of the feet.

Twelve chondrosarcomas of the bones of the feet from 11 patients in the Scottish Bone Tumor Registry were reviewed. One patient with diaphyseal aclasis (osteochondromatosis) developed 2 chondrosarcomas. The mean age of patients was 52.3 years (range, 17 to 83 years). Men were predominantly affected. Four tumors affected the tarsal bones; the rest involved the short tubular bones. The usual clinical presentation was a painful, progressively enlarging swelling. Radiologically, most showed some bone expansion, cortical destruction with indistinct margins, and soft-tissue extension. Histologically, the majority were middle-grade tumors. Treatment included curettage or local excision for 4 tumors and amputation or ray resection for 8 tumors. Follow-up varied from 6 months to 18 years (average, 5.8 years). Local recurrence after surgery was seen in 3 patients. All 3 died because of metastases to the lungs or brain.

Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes share a common t(7;16)(q34;p11) translocation.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare metastasizing soft tissue tumor with deceptively bland histologic features. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is thought to be a closely related tumor differing only by the presence of collagen rosettes. We report the occurrence of a common t(7;16)(q34;p11) translocation in 2 cases of HSCT and 2 cases of LGFMS, thereby providing the first cytogenetic proof that LGFMS and HSCT are variants of the same entity. The tumors occurred in the thighs of 2 females and in the buttock and supraclavicular fossa of 2 males. One HSCT had a spectrum of unusual histologic features, including the presence of plump epithelioid cells with abundant cytoplasm and strands and nests of clear epithelioid cells separated by eosinophilic hyalinized stroma. Two cases showed a hitherto unreported, focal staining with epithelial membrane antigen, thus adding to the immunohistochemical profile of these tumors. LGFMS and HSCT probably have a wider spectrum of morphologic features than previously thought, the awareness of which will help pathologists to avoid diagnostic pitfalls. Demonstration of the t(7;16)(q34;p11) translocation will help to diagnose difficult cases with unusual histologic features.

Chondroblastoma: varied histologic appearance, potential diagnostic pitfalls, and clinicopathologic features associated with local recurrence.

Chondroblastoma is a rare, benign bone tumor. Although it has distinctive clinicopathologic features, its wide morphologic spectrum may pose diagnostic problems. We present the clinicopathologic features of 42 patients (28 males, 14 females; age range, 8 to 66 years), with emphasis on unusual histologic features, potential diagnostic pitfalls, and factors associated with recurrence. Thirty-four tumors were in long bones, with the most common site being the proximal femur. Unusual histologic features included the presence of atypical, epithelioid, spindle, and foamy cells and necrosis and a diffuse basophilic myxoid matrix. Tumors with focal osteoclast-like giant cell rich areas (n = 11), prominent cystic change (n = 8) and extensive fibromyxoid areas (n = 3) resembled giant cell tumors, aneurysmal bone cysts, and chondromyxoid fibromas, respectively. The diagnosis of referring pathologists was inaccurate in 34% of cases. Six patients (14%) had local recurrence. The only clinical feature significantly associated with increased risk of local recurrence was duration of symptoms for less than 6 months (log rank P =.003). None of the histologic features was significantly associated with recurrence. These included worrisome features such as cellular atypia, necrosis, and mitoses. None of the patients had metastases. An increased awareness of the morphologic spectrum of chondroblastomas will enable pathologists to avoid diagnostic pitfalls. We emphasize the need for a combined clinical, radiologic and histologic approach to the diagnosis of chondroblastomas.

Mid-trimester fracture of femur in a normal fetus.

We report a case of an intrauterine fracture of the femur detected at routine mid-trimester sonography in an otherwise normal fetus. There was no associated maternal trauma. At birth, callus formation was palpable and confirmed by radiography. Absence of further fractures despite normal childhood activity and other features precluded a diagnosis of skeletal dysplasia. Although such an event is extremely rare, the possibility of an intrauterine origin should be considered in the differential diagnosis of battered baby syndrome presenting with a single fracture.

Premonitory Pain Preceding Swelling: A Distinctive Clinical Presentation of Synovial Sarcoma which may Prompt Early Detection.

PURPOSE: The aim of this paper is to document the unusual presentation of long-standing pain at the tumour site before development of a swelling in patients with synovial sarcoma.Patients/methods and results: The clinical presentation of 53 patients with synovial sarcoma was compared with 56 randomly selected patients with other sarcomas of the trunk and extremities. The two groups were similar with regard to age (P = 0.980), sex (P = 0.784) duration of symptoms (P = 0.697), size (P = 0.931) and site of tumour (P = 0.288). Sixteen (30.2%) patients with synovial sarcoma had pain before development of a swelling compared to two (3.6%) patients with other sarcomas (P < 0.001, odds ratio = 11.68, 95% confidence interval 2.53, 53.83). The mean duration of such pain was 37 months (median 24, range 6-120 months). The nature of the pain was variable. Eight patients had sharply localised tenderness. Calcification seen in the X-rays of four patients was initially misdiagnosed as benign lesions. A swelling was ultimately detected by MRI, CT, ultrasound or at physical examination. The mean duration from first presentation with pain till diagnosis of synovial sarcoma was 20 months. In three patients, at explorative surgery there was friable, vascular or necrotic tissue in the absence of a well-defined tumour mass. DISCUSSION: The occurrence of long-standing pain at the tumour site prior to development of a swelling is significantly more common with synovial sarcomas than with other sarcomas. Awareness of this unusual presentation and appropriate investigation may enable detection of synovial sarcoma at a prognostically favourable early stage.