A rare case of aggressive primary pulmonary synovial sarcoma: A case report

Synovial sarcoma is a malignant mesenchymal tumour which mostly affects young adults and is mostly seen in extremities. Primary synovial sarcoma arising from the lung is rare, accounting for less than 0.5% of all lung carcinomas. Most commonly it arises from the lung followed by pleura and mediastinum. Primary pulmonary synovial sarcoma is an extremely aggressive malignant tumour that can invade adjacent organs and give distant metastasis. Histologically it is of two main types – monophasic and biphasic. IHC is a must for diagnosis following clinical examination and imaging. Here we report a case of an elderly male with right lung mass lesion infiltrating the visceral and mediastinal pleura. PET – computerized tomography (CT) guided biopsy was s/o synovial sarcoma monophasic type which was further confirmed by IHC. The multimodality treated for this includes wide resection, chemotherapy and radiotherapy. Synovial sarcoma is relatively chemosensitive though it is considered as a high grade tumour with a poor prognosis. Because of the advanced stage of the disease our patient was not a candidate for surgery and was taken up for chemotherapy. He had a survival of 6 months but had succumbed due to non – cancer related cause.

Il craniofaringioma

Craniopharyngioma (CP) is a rare epithelial low-grade tumour that develops in the sellar/suprasellar region of the brain, along the craniopharyngeal duct. It has a bimodal distribution and the first peak occurs in paediatric age almost exclusively consisting of the adamantinomatous subtype. A second peak occurs in adulthood after the fifth decade of life and is more likely to be of the papillary subtype. Therapy is based on surgical removal, so the best approach is the endonasal approach, which is sometimes associated with radiation therapy. Molecular target drugs are a promising novelty, indeed they are already in use in adults and are being tested in children. Although CP is considered a low malignancy tumour, its localization and close relationships with important structures such as the optical pathways, the hypothalamic-pituitary axis and the thalamus burden it with important complications (visual disturbances, central obesity, dysendocrinopathies) that can interfere with the patient’s quality of life.

Impact of NICE guidelines on the survival of patients with soft-tissue sarcomas

Aims Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS. Methods A total of 2,427 patients with an STS referred to a supraregional centre in the ten-year periods before (n = 1,386) and after (n = 1,041) the issue of the NICE guidelines were evaluated. Results The mean size of the tumour was significantly smaller at the time of diagnosis (10.3 cm (SD 6.5) vs 9.1 cm (SD 6.2); p < 0.001) and the number of patients who had undergone an inadvertent excision significantly decreased (28% (n = 389) vs 20% (n = 204); p < 0.001) following the introduction of the NICE guidelines. The five-year DSS was 63% in the pre-NICE and 71% in post-NICE groups (p < 0.001). The improved survival was more significant for those with a high-grade tumour (pre-NICE, 48%; post-NICE, 68%; p < 0.001). In those with a high-grade tumour, the mean size of the tumour (11.6 cm (SD 6.2) vs 9.6 cm (SD 5.8); p < 0.001) and the number of patients with metastasis at the time of diagnosis (15% (n = 124 vs 10% (n = 80); p = 0.007) significantly decreased in the post-NICE group. Conclusion An improvement in survival was seen after the introduction of the NICE guidelines, especially in patients with a high-grade STS. More patients were referred at an earlier stage, indicating a clearer pathway after the issue of national policy for the management of STSs in the UK. Cite this article: Bone Joint J 2021;103-B(3):569–577.

Primary cutaneous mucinous carcinoma of axilla: a case report with review of literature

Primary cutaneous mucinous carcinoma is a low grade tumour of the sweat gland rarely occurring in middle aged men, more commonly in the sixth decade. When seen in a younger age group, the tumour tends to be more aggressive with potential for metastasis. Clinical presentation is usually a hard nodular well defined painless lesion confined to skin and subcutaneous tissue, commonly seen in head and neck region. Histopathological examination of the specimen shows cells with myxoid and mucinous material, confirmed by immunohistochemistry showing positive for estrogens and progesterone receptor and cytokeratin 7, is diagnostic. Surgery is the treatment of choice, wide local excision with margin of 1 cm as this tumor is resistant to radiotherapy and chemotherapy. Its tendency for late recurrences and rare metastasis, adds to the good prognosis. With rare chances of distant metastasis, prognosis remains good but local recurrences are not uncommon after excision.

Clinical Implications of (Pro)renin Receptor (PRR) Expression in Renal Tumours

(1) Background: Renal cancer is one of the most frequent malignancies in Western countries, with an unpredictable clinical outcome, partly due to its high heterogeneity and the scarcity of reliable biomarkers of tumour progression. (Pro)renin receptor (PRR) is a novel receptor of the renin–angiotensin system (RAS) that has been associated with the development and progression of some solid tumours by RAS-dependent and -independent mechanisms. (2) Methods: In this study, we analysed the immunohistochemical expression of PRR at the centre and border in a series of 83 clear-cell renal cell (CCRCCs), 19 papillary (PRCC) and 7 chromophobe (ChRCC) renal cell carcinomas, and the benign tumour renal oncocytoma (RO, n = 11). (3) Results: PRR is expressed in all the tumour subtypes, with higher mean staining intensity in ChRCCs and ROs. A high expression of PRR at the tumour centre and at the infiltrative front of CCRCC tissues is significantly associated with high grade, tumour diameter, local invasion and stage, and with high mortality risk by UCLA integrated staging system (UISS) scale. (4) Conclusions: These findings indicate that PRR is associated with the development and progression of renal tumours. Its potential as a novel biomarker for RCC diagnosis/prognosis and as a promising therapeutic target should be taken into account in the future.

In modern times, how important are breast cancer stage, grade and receptor subtype for survival: a population-based cohort study

Background In breast cancer, immunohistochemistry (IHC) subtypes, together with grade and stage, are well-known independent predictors of breast cancer death. Given the immense changes in breast cancer treatment and survival over time, we used recent population-based data to test the combined influence of IHC subtypes, grade and stage on breast cancer death. Methods We identified 24,137 women with invasive breast cancer aged 20 to 74 between 2005 and 2015 in the database of the Cancer Registry of Norway. Kaplan-Meier curves, mortality rates and adjusted hazard ratios for breast cancer death were estimated by IHC subtypes, grade, tumour size and nodal status during 13 years of follow-up. Results Within all IHC subtypes, grade, tumour size and nodal status were independent predictors of breast cancer death. When combining all prognostic factors, the risk of death was 20- to 40-fold higher in the worst groups compared to the group with the smallest size, low grade and ER+PR+HER2− status. Among node-negative ER+HER2− tumours, larger size conferred a significantly increased breast cancer mortality. ER+PR−HER2− tumours of high grade and advanced stage showed particularly high breast cancer mortality similar to TNBC. When examining early versus late mortality, grade, size and nodal status explained most of the late (> 5 years) mortality among ER+ subtypes. Conclusions There is a wide range of risks of dying from breast cancer, also across small breast tumours of low/intermediate grade, and among node-negative tumours. Thus, even with modern breast cancer treatment, stage, grade and molecular subtype (reflected by IHC subtypes) matter for prognosis.

Clinicopathological profile of invasive ductal breast carcinoma in a government tertiary care center in eastern India

Introduction and Aim: There is an increasing incidence of breast cancer (BC) in our country. The study aimed to find out the demographic profile, clinical presentation, and management in patients treated for invasive ductal breast carcinoma (IDC) in a rural government teaching hospital and to study the association of sociodemographic factors with BC stage. Materials and Methods: The study retrospectively analyzed 100 IDC females treated at Midnapore medical college, West Bengal, India from January 2017 to December 2019. The study included female patients of all ages diagnosed with IDC who had undergone surgery. Male BC, bilateral BC, other pathological types of BC were excluded. Results: The mean age was 56.54±12.99 years. Sixty-seven cases were postmenopausal. Eighty-one cases were from rural areas. Seventy-three cases had education up to middle school. Eighty-six cases had a lower socioeconomic status. Ninety-eight cases had unilateral breast lump and only 2% had a nonpalpable breast lump. The left breast was more commonly involved. The mean tumour size was 4.33 cm. Ninety-eight cases underwent a modified radical mastectomy. Stage III was the most commonly observed BC and seen in 40 cases followed by stage II in 36 cases. Sixty-one cases had advanced BC. Liver metastasis was seen in 21cases. Sixty-nine cases had axillary node(ALN) positivity. Sixty-eight cases had lymphovascular emboli (LVE). High-grade tumour was seen in 77%. High-grade tumour was more common in young women. Seventy three cases of tumours were estrogen receptor-positive (ER+) while 66% progesterone receptor-positive (PR+). Eighteen cases were human epidermal growth factor 2 receptor-positive. Conclusion: High incidence of advanced BC is found in rural areas due to a lack of awareness and health infrastructure.

Co-targeting PIM and PI3K/mTOR using multikinase inhibitor AUM302 and a combination of AZD-1208 and BEZ235 in prostate cancer

PIM and PI3K/mTOR pathways are often dysregulated in prostate cancer, and may lead to decreased survival, increased metastasis and invasion. The pathways are heavily interconnected and act on a variety of common effectors that can lead to the development of resistance to drug inhibitors. Most current treatments exhibit issues with toxicity and resistance. We investigated the novel multikinase PIM/PI3K/mTOR inhibitor, AUM302, versus a combination of the PIM inhibitor, AZD-1208, and the PI3K/mTOR inhibitor BEZ235 (Dactolisib) to determine their impact on mRNA and phosphoprotein expression, as well as their functional efficacy. We have determined that around 20% of prostate cancer patients overexpress the direct targets of these drugs, and this cohort are more likely to have a high Gleason grade tumour (≥ Gleason 8). A co-targeted inhibition approach offered broader inhibition of genes and phosphoproteins in the PI3K/mTOR pathway, when compared to single kinase inhibition. The preclinical inhibitor AUM302, used at a lower dose, elicited a comparable or superior functional outcome compared with combined AZD-1208 + BEZ235, which have been investigated in clinical trials, and could help to reduce treatment toxicity in future trials. We believe that a co-targeting approach is a viable therapeutic strategy that should be developed further in pre-clinical studies.