Giant fibroepithelial polyp of vulva with fibroid uterus with primary lymphedema (Meige disease/lymphedema praecox): a rare case report

Fibroepithelial polyps or FSPs develop in the reproductive years of young to middle-aged women. They primarily affect the vagina, and their occurrence in the vulva is less common. Giant FSP are very rare. We present a case of a patient with giant FSP of the vulva with unilateral lymphedema. A forty year old patient presented with history of mild, dull, aching, continuous pain in lower abdomen. After her first delivery, she had developed pruritis over vulva followed by appearance of a growth. A non-tender, mobile warty growth was found on the examination of vulva, and it involved the mons pubis, clitoris and left labia minora and major. Dermatological consultation made a preliminary diagnosis of nevoid growth vulva. Operative findings from exploratory laparotomy confirmed that uterus had enlarged to 28 weeks size. The diagnosis of fibroepithelial polyp was confirmed by histopathological examination. A common pathogenetic background of lymphedematous FSPs is persistent lymph stasis, along with consequent injury of microcirculation and stromal hyperplasia. The role of hormonal factors in the development of these lesions in also supported by the Estrogen and progesterone receptor positivity. The association of FSPs with unilateral lymphedema, as in our patient, is very rare. This case provides some evidence that chronic lymph stasis can lead to microcirculation injury which further lead to stromal hyperplasia.

Does Concomitant DCIS Affect the Clinical Outcome in Breast Cancer Patients with Invasive Ductal Carcinoma: an Asian Perspective

BackgroundDuctal carcinoma in situ (DCIS) is an established precursor to invasive ductal carcinoma (IDC) and its coexistence with IDC appear to favour reduced biological aggressiveness. Its prognostic implication and ability to affect clinical outcome has been understudied in Asia. This study aims to explore if concomitant DCIS affects the clinical behavior and outcomes among Asians. MethodsStage I to III breast cancer patients with histologically proven invasive ductal carcinoma, diagnosed and treated in a single institution from 1 June 2004 to 30 June 2014 were included in this study. Statistical analyses were conducted using x2 test, independent T- test, multi-variate logistic regression and Kaplan- Meier test.ResultsA total of 818 patients were identified, including 224 and 594 patients with isolated IDC (No-DCIS) and IDC with coexisting DCIS (IDC-DCIS) respectively. Patients with IDC-DCIS were found to have smaller tumours (median: 22mm, p<=0.01), estrogen receptor positivity (p=0.001), progesterone receptor positivity (p<0.001) and associated with better pathological stage (p=0.001). Patients with No-DCIS were 1.6 times more likely to develop disease progression (95% CI: 1.1- 2.3, p=0.027) and subsequently associated with distant recurrences (20.5% vs 13.6%, p=0.02). The breast cancer specific 5 year overall survival rate for patients with No-DCIS and those with IDC-DCIS was 90.9% (95% CI: 86.2% - 94.5%) and 93.7% (95% CI: 91.4- 95.5%) respectively (p=0.202).ConclusionThe presence of DCIS component in IDC among Asians is associated with favourable tumour biological profile, thereby indicating reduced disease aggressiveness. Our study is the first to report the clinical significance in terms of disease progression and distant recurrences among Asians. Trial registrationThis study had received the approval of the institutional ethics committee prior to its commencement (IRB Ref No: 2019/2884).

Uterine intravenous leiomyomatosis with femoral vein, intracaval, intracardiac and pulmonary artery extension

We report a 54-year-old female with recently diagnosed multiple cardiac thrombi and pulmonary embolism that was treated with thrombolytics and anticoagulants. She presented again with worsening dyspnea and was found to have persistent large cardiac thrombi on echocardiogram. Surgical findings revealed a single right atrial mass originating from inferior vena cava and extending into the pulmonary artery. The mass was successfully removed. Final pathology revealed a benign smooth muscle and vascular mass with estrogen and progesterone receptor positivity favoring uterine intravenous leiomyoma. She was discharged on warfarin and leuprolide therapy. This diagnosis requires a high index of suspicion, especially in a middle-aged woman with right atrial mass and history of an existing leiomyoma, hysterectomy or myomectomy.

Common matrix metalloproteinase-2 gene variants and altered susceptibility to breast cancer and associated features in Tunisian women

A role for matrix metalloproteinase polymorphisms in breast cancer development and progression was proposed, but with inconclusive results. We assessed the relation of matrix metalloproteinase-2 variants with breast cancer and related phenotypes in Tunisians. This case-control retrospective study involved 430 women with breast cancer and 498 healthy controls. Genotyping of matrix metalloproteinase-2 rs243866, rs243865, rs243864, and rs2285053 was analyzed by allelic exclusion. The minor allele frequency of rs2285053 was significantly lower in women with breast cancer cases as compared to control women; minor allele frequencies of the remaining single-nucleotide polymorphisms were similar between cases and control women. The distribution of rs243865 and rs2285053 genotypes was significantly different between breast cancer patients and control subjects. This persisted when key covariates were controlled for. None of the matrix metalloproteinase-2 variants were associated with estrogen receptor positivity, progesterone receptor positivity, or with double estrogen receptor–progesterone receptor positivity in breast cancer patients. Matrix metalloproteinase-2 rs243866, rs243865, and rs243864 were positively associated with menstrual irregularity and histological type, while rs243866 and rs2285053 were negatively associated with menarche and nodal status. In addition, rs2285053 was negatively associated with triple negativity, tumor size, distance metastasis, molecular type, and chemotherapy. Haploview analysis revealed high linkage disequilibrium between matrix metalloproteinase-2 variants. Four-locus Haploview analysis identified haplotypes GCTT and GTTC to be negatively associated with breast cancer, which remained statistically after controlling for key covariates. Matrix metalloproteinase-2 alleles and genotypes, along with four-locus haplotypes, are related to reduced susceptibility to breast cancer in Tunisian women, suggesting a protective effect.

A Rare Cause of Stroke in Young Adults: Occlusion of the Middle Cerebral Artery by a Meningioma Postpartum

Meningioma is the most common nonglial intracranial primary tumor. It is a slowly growing tumor and presents clinically by causing seizures along with neurological or neuropsychological deficit. However, acute presentation of meningioma is possible. We are reporting a case of cerebral infarction due to a sphenoid wing meningothelial meningioma (with progesterone receptor positivity) leading to an occlusion of the middle cerebral artery (MCA) in a 30-year-old right-handed woman (1 month after childbirth). After surgery, no new neurological event occurred, and she recovered most of her neurological functions. Strokes due to meningioma are a highly rare clinical occurrence but should be given serious consideration, particularly in young patients.

Revision 2: an immunohistochemical approach and evaluation of solid pseudopapillary tumour of the pancreas

Solid pseudopapillary tumours (SPT) of the pancreas are uncommon, but with widespread and increased imaging, several of these lesions are coming to light incidentally and are subject to needle biopsies. On limited material and especially the solid or clear cell, variants of SPT can morphologically mimic most notably pancreatic neuroendocrine tumours and even metastatic renal cell carcinoma or melanoma. In this context, immunohistochemistry is important and useful in helping to reach the correct diagnosis. Several antibodies have been used in the immunohistochemical evaluation of SPT. As with most tumours, no one marker is specific, but rather a core panel is advocated. Recently, both β-catenin and E-cadherin have been shown to be of value in SPT. Nuclear and cytoplasmic decoration of tumour cells by β-catenin is seen in almost 100% of cases. This protein relocalisation away from the cell membrane is underscored by mutations of the β-catenin gene. Mutations of the CDH1 gene are very uncommon in SPT, but the immunohistochemically detected changes to the protein are consistent and present in 100% of cases. Using an E-cadherin antibody to the extracellular domain of the molecule results in complete membrane loss, while the antibody directed to the cytoplasmic fragment produces distinct nuclear staining of the tumour cells. In addition, there is concordance of staining abnormalities between the two antibodies. When combined with CD10 and progesterone receptor positivity, a diagnosis of SPT can be rendered with confidence even in small biopsy samples.