Abir Karoui*, Ahmed Cherif, Olfa Chaffai, Wassim Saidi, Ghada Sahraoui, Sana Menjli, Mohamed Badis Chanoufi, Nadia Boujelbene and Hssine Saber Abouda
Published on: 2nd September, 2022
Background: Leiomyomas beyond the uterus are defined by benign smooth muscle cell tumors outside of the uterus. Intravenous leiomyomatosis is a rare type of uterine leiomyoma and is characterized by the formation and growth of benign leiomyoma tissue within the vascular wall. Herein, we present a case of Intravenous leiomyomatosis successfully treated by surgical removal and a review of actual medical recommendations.Case presentation: A 49 - year-old woman, maghrébin, G3 P2, no family history of uterine myomas mentioned, having systemic arterial hypertension, presented to our department with hypogastric pain and abnormal uterine bleeding in the prior five months resulting in anemia which required iron supplementation. On physical examination the vital signs were normal. A palpable mass in the hypogastrium was noted. The rest of the exam was unremarkable. Pelvic ultrasound showed a huge uterus with multiple heterogeneous leiomyomas, including at least one intracavity. Computed tomography scans and magnetic resonance imaging were not done initially due to the unaffordability of the patient. The initial diagnosis was leiomyoma. The decision to perform a total abdominal hysterectomy and bilateral salpingo-oophorectomy was taken. The abdomen was opened by a midline vertical incision. During surgery, multiple subserosal, intramural and submucosal fibroids ranging from 2 cm × 3 cm to 10 cm × 10 cm were seen. On pathological examination, the uterus measured 19 cm in the largest diameter and weighed 1.3 kg. The cut section showed white nodular myometrial masses. Microscopically, intravascular growth of benign smooth muscle cells is found within venous channels lined by endothelium. The diagnosis of Intravenous leiomyomatosis of the uterus without malignant transformation was retained. The patient was monitored for 14 months and subsequent computed tomography did not reveal any evidence of tumor recurrence. The follow-up will be performed annually till the age of menopause.Conclusion: Intravenous leiomyomatosis is a benign, rare and potentially lethal pathology. It especially affects premenopausal women with a history of uterine myoma, whether operated on or not. They require close and prolonged follow-up because of the high risk of recurrence.
Nida Mishraz Siddiqui*, Kapila Hari, Bilal Bobat, Dinen Parbhoo, Vikash Lala and Adam Mahomed
Published on: 31st December, 2022
Background: Liver Transplantation (LT) is the definitive treatment for Autoimmune Hepatitis (AIH) in patients with decompensated cirrhosis, liver failure and hepatocellular carcinoma. Outcomes of LT in AIH among black-Africans are not well-defined. We performed a single-center retrospective-review of adult LT patients. The study period was from 1st August 2004-31st August 2019. The primary aim was to document 1- & 5- year patient and graft survival. A secondary aim was to compare the survival of black-Africans to Caucasians. Data was analyzed using survival-analysis. Results: A total of 56 LT were performed for AIH. Sixty-seven percent (n = 38/56) had confirmed AIH on explant histology. Of these, the majority i.e., 79% (30/38) were female and 21% (8/38) were male. There were equal numbers of black-African 42% (n = 16/38) and Caucasian 42% (n = 16/38) patients. Rejection was four-times higher in black-Africans as compared to Caucasians. Forty-four percent (n = 17/38) had an acute rejection episode and 13% (5/38) had chronic rejection. Recurrence was found in four black-African females. Post-LT patient survival at 1- and 5- years was 86.5% and 80.7%, and graft survival was 94% and 70.8% respectively. The 5- year patient survival was insignificantly lower for black-Africans (73.9%) as compared to Caucasians (83.7%) (p - value 0.26, CI 6.3 - 12.2). Five-year graft survival was significantly lower among black-Africans (55%) as compared to Caucasians (84.8%) (p - value 0.003 CI 3.8 - 8.1)Conclusion: Black-Africans had a four-fold higher rate of rejection compared to Caucasians. Recurrent AIH was only found in patients of black ethnicity. Similar 1- & 5- year patient survival rates were observed between the two ethnicities. The 5-year graft survival among black-Africans was significantly lower than Caucasians.
Raheel Khan, Atiq-ur-Rehman Selehria, Hafsa Aquil, Atif Sheraz, Sara Khan, Najwa Zahoor and Anashia Kayani*
Published on: 10th April, 2023
Gliomas are known to be one of the most grievous malignant central nervous system (CNS) tumors and have a high mortality rate with a low survival rate severe disability and increase risk of recurrence. Aim of his study is to determine the diagnostic accuracy of apparent diffusion coefficient (ADC) in differentiating low-grade and high-grade gliomas, taking histopathology as the gold standard. It is a Cross-sectional validation study conducted at the Armed Forces Institute of Radiology and Imaging, (AFIRI) Rawalpindi, Pakistan from 28th February 2022 to 27th August 2022.Materials and methods: A total of 215 patients with focal brain lesions of age 25-65 years of either gender were included. Patients with a cardiac pacemaker, breastfeeding females, de-myelinating lesions and malignant infiltrates, and renal failure were excluded. Then diffusion-weighted magnetic resonance imaging was performed on each patient by using a 1.5 Tesla MR system. The area of greatest diffusion restriction (lowest ADC) within the solid tumor component was identified while avoiding areas of peritumoral edema. Results of ADC were interpreted by a consultant radiologist (at least 5 years of post-fellowship experience) for high or low-grade glioma. After this, each patient has undergone a biopsy in the concerned ward, and histopathology results were compared with ADC findings. Results: Overall sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of apparent diffusion coefficient (ADC) in differentiating low- and high-grade gliomas, taking histopathology as the gold standard was 93.65%, 87.64%, 91.47%, 90.70% and 91.16% respectively. Conclusion: This study concluded that apparent diffusion coefficient (ADC) is the non-invasive modality of choice with high diagnostic accuracy in differentiating low- and high-grade gliomas.
Raul F Valenzuela*, Behrang Amini, Elvis Duran-Sierra, MA Canjirathinkal, John E Madewell, Colleen M Costelloe and William A Murphy
Published on: 20th September, 2023
Soft-tissue sarcomas are a rare and complex group of malignant tumors. Advanced MRI sequences such as diffusion-weighted imaging (DWI) and perfusion-weighted imaging/dynamic contrast enhancement (PWI/DCE) can provide valuable tumor characterization and treatment response assessment. In the case of archetypical cellular tumors such as Pleomorphic Undifferentiated sarcoma (UPS), Good responders often display right-side displacement of the ADC intensity histogram, resulting in increased ADC-mean and decreased kurtosis and Skewness compared with Baseline and poor responders’ more left-sided curve. The PWI/DCE pattern most often associated with a good response is the presence of a “capsular-like” enhancement and a TIC type 2. Sarcoma hemorrhage patterns on SWI emerge during treatment, including “interstitial,” globular,” “luminal,” and incomplete and complete “peripheral ring-like” tumor wall hemosiderin impregnation. Treatment-induced bleeding is typically associated with low SWI-mean values and a left-sided intensity histogram with positive Skewness.During post-surgical surveillance, DCE MR imaging can reliably distinguish recurrent sarcoma from post-surgical scarring. TICs III, IV, and V raise the suspicion of local tumor recurrence, while TIC type II usually represents benign post-operative change such as granulation tissue. Advanced MRI is an essential tool for assessing sarcomas during and after therapy.
Govani DJ, Zaparackaite I, Singh SJ, Bhattacharya D, Swamy KB, Correia RC, Midha PK and Patel RV*
Published on: 20th December, 2023
A very unusual, interesting, and challenging case of a 24-year-old female who was born with three openings in the neck. The patient had chronic abdominal gaseous distention, recurrent abdominal pain, and constipation since early infancy. The patient presented in emergency with acute painful red, hot, and tender swelling in the left upper cervical area. Laboratory studies showed high inflammatory markers and a provisional diagnosis of abscess with a sinus was made. The patient underwent an emergency incision and drainage. Sinus recurred and a sinogram showed it to be a residual cyst in the left submandibular salivary gland. The total cyst excision was attempted with resultant recurrence and grade IV facial nerve palsy. Post-operatively recurrent infections caused by Methicillin-resistant Staphylococcus aureus (MRSA) required several courses of oral and intravenous broad-spectrum antibiotics with several hospital admissions with no resolution in sight. Subsequent ultrasound and magnetic resonance imaging showed a residual infected cyst, cutaneous sinus, and a fistula opening in the left ear canal. A diagnosis of branchial cyst type II of the first brachial cleft remnant with a fistula was established with bilateral branchial fistulas of the second branchial remnants and the associated colorectal hypoganglionosis based on radiological studies. The patient refused any further operative interventions. Therefore, the option of conservative treatment of hypoganglionosis with holobiotics consisting of prebiotics, probiotics and postbiotics, laxatives, dietary changes, lifestyle modifications, and dietary supplements started. All antibiotics were stopped. These therapies resulted in the resolution of residual first branchial remnants and recurrent MRSA infections with the improvement in the facial nerve palsy from grade V to grade III-IV together with an excellent cosmetic and functional result. The patient is doing well at follow-ups being infection-free for 18 months and repeat contrast-enhanced computed tomogram (CECT) has shown complete resolution of the residual cyst, sinus, and fistula with fibrosis.
Background: Endometrial morular metaplasia, a clinical conundrum from a diagnostic and management angle given its rarity and low oncogenic potential, has been linked to endometrial hyperplasia and carcinoma. Case report: A 77-year-old woman with no significant past medical history, was found to have an asymptomatic thickened endometrium on pelvic imaging, after presenting with lower abdominal pain, 3yrs ago. Diagnostic hysteroscopy identified an endometrial polyp within a pyometra. Histopathology showed focal complex endometrial hyperplasia without atypia with superimposed morular metaplasia(EMM) with a negative microbiology assay.Following conservative management with multidisciplinary team(MDT) overview, as-per patient choice with 6-monthly follow-up hysteroscopy, endometrial biopsies and a short use of the Mirena® Intrauterine system (discontinued following poor tolerance), histopathology shows resolved hyperplasia with persistent EMM. Due to persistent disease, a hysterectomy is under consideration.Discussion: Current evidence suggests that a sub-type of EMM, a likely histological manifestation of beta-catenin (CTNNB1) gene mutation: could be a precursor of endometrial hyperplasia and low-grade endometrioid-endometrial carcinoma sub-type. Though low-grade in nature, the increased recurrence risk raises significant concerns.Prognostication following gene mutation identification can help with management options which include conservative, hormonal therapy with adjunct repeat endometrial sampling: or hysterectomy. The optimal frequency of endometrial sampling when uterine-sparing, is unclear, leading to a management conundrum, whilst persistent disease may require a hysterectomy.Conclusion: Management of endometrial morular metaplasia can be difficult but must reflect the woman’s choice with a MDT-overview. Immuno-histochemical tools utilizing new molecular biological advances, can simplify the diagnostic and prognostication processes, aiding clinical management.
Mehdi Marrak*, Yassine Ouanes, Kays Chaker, Moez Rahoui, Mokhtar Bibi and Yassine Nouira
Published on: 13th May, 2024
An inverted papilloma of the urinary tract is a rare benign lesion. A 35-year-old male presented gross hematuria. Cystoscopy showed one, papillary tumor at the bladder trigone. Transurethral resection was done, and histological examination has concluded in the diagnosis of inverted papilloma. Following resection, the patient was asymptomatic with no hematuria and no recurrence after a five-year cystoscopy control. We report a case of bladder Inverted papilloma and we aim to remind the clinical, histological, and therapeutic features of this rare tumor.
Alia Mousli, Mohamed Aziz Cherif*, Marouen Benna, Ameni Yousfi, Semia Zaraa, Rim Abidi and Chiraz Nasr
Published on: 8th July, 2024
Background: Tailored adjuvant treatment is key to managing endometrial cancer effectively. Understanding prognostic factors of loco-regional failure and the impact of adjuvant treatment can help in treatment de-escalation without compromising survival outcomes.The aim of this study was to assess the pattern of failure in endometrial cancer patients and to determine predicting Loco-Regional Recurrence (LRR) factors.Patients and methods: Data were collected from 214 patients treated for endometrial cancer between 2005 and 2012 in Salah Azaiez Institute in Tunisia. All patients underwent upfront surgery followed by adjuvant brachytherapy with or without external beam radiation. The median follow-up period was 44 months. Univariate and multivariate analyses were performed to identify prognostic factors for LRR.Results: The 5-year overall survival rate was 78.1%, and the 5-year progression-free survival rate was 80.1%. LRR occurred in 25 patients (11.6%), with a median recurrence time of 29 months (range 4 months - 46 months). Pelvic relapse was the most common site, occurring in 10 patients. Vaginal relapses were observed in 9 patients, and retro-peritoneal relapses were observed in 6 cases. FIGO stage, tumor grade, histologic type, Lympho-Vascular Space Invasion (LVSI), and delays in adjuvant treatment were significant predictors of LRR.Conclusion: Identifying prognostic factors for LRR in endometrial cancer is crucial for optimizing adjuvant treatment strategies. Higher FIGO stages and the presence of LVSI were independent predictive factors for LRR. Tailored adjuvant treatment, taking these prognostic factors into account, is essential to improve patient outcomes and minimize unnecessary treatment-related toxicity.
Giovanni Martino*, Mario Leporace*, Francesco Greco, Rossella Quarta, Letizia Romano, Antonio Curcio and Alberto Polimeni
Published on: 8th August, 2024
Spontaneous Coronary Artery Dissection (SCAD) represents a significant cause of acute coronary syndrome (ACS) in patient populations with low-risk cardiovascular profiles and, therefore can be sometimes underdiagnosed. On the other hand, it often preserves the typical clinical presentation of ACS which makes coronary artery angiography (CAG) execution mandatory. A 62-year-old woman with a history of recurrent SCAD presented to the emergency department for a new episode of acute chest pain with troponin elevation. CAG revealed an ambiguous angiography image suggesting a sub-occlusive type 2 SCAD involving the distal segment of the left circumflex artery. The patient was managed conservatively due to the absence of ongoing ischemia or hemodynamic instability. At the seven-day follow-up, a computed tomography coronary angiography (CTCA) was performed to better assess SCAD and detect concomitant associated arteriopathies. Optimized medical therapy was prescribed at the discharge and at one-month follow-up, no recurrence of symptoms was referred.
Cholangiocarcinoma (CCA), a rare malignancy originating from bile duct epithelial cells, often presents a challenging prognosis due to its rarity, delayed diagnosis, and early recurrence post-curative-intent treatments. Additional complexities include difficulties in achieving R0 resection during surgical intervention and the lack of effective second-line treatments following the failure of first-line regimens, particularly in unresectable advanced cases.In this case study, we demonstrate a durable response to a combination regimen of pembrolizumab and lenvatinib in a patient with distal CCA. Despite the regimen’s interim median Progression-Free Survival (PFS) of 6.1 months (95% CI, 2.1-6.4), our patient achieved a clinical and radiological PFS of approximately two years. The underlying mechanisms, potentially involving the upregulation of immune response pathways through undisclosed means or influenced by lenvatinib’s activation of T cells, might augment the sensitivity to PD-1 antibodies like pembrolizumab, contributing to the patient’s sustained response over two years.This case also highlights the significance of the patient’s initial good health condition, multidisciplinary care, and the potential impact of molecular subtyping on treatment selection in a patient with distal CCA who underwent numerous diagnostic procedures, intricate surgical interventions, and subsequent treatment regimens over seven years. Additionally, we underscore significant landmark trials and emerging combination therapies, including chemotherapies, immunotherapy, and targeted treatments in this report.
Gizem Kavak, Mehmet Ali Nahit Şendur, Dilek Kösehan, Ayşen Aydin, Mehmet Ferat Kiran and Yildiz Güney*
Published on: 27th January, 2025
Anaplastic Pleomorphic Xanthoastrocytoma is a very rare tumor of the Central Nervous System (CNS). BRAFV600E mutation is a common mutation in pleomorphic xanthoastrocytoma. We present a 52-year-old male patient who underwent total resection due to a temporal lobe mass. The primary tumor in the temporal lobe was given postoperative radiotherapy. During follow-up, genetically and histologically proven metastasis was detected in the paraspinal region. The adjuvant RT was given to metastasis after surgery. The patient, who used the combination of dabrafenib and trametinib after recurrence, is being monitored in remission.
Oumaima Fakir*, Hanaa Lazhar, Aziz Slaoui, Amina Lakhdar and Aziz Baydada
Published on: 7th March, 2025
Bartholinitis, or Bartholin's gland abscess, is a relatively common gynecological condition among women of reproductive age. Its annual incidence is estimated at approximately 0.5 per 1,000 women, which corresponds to a lifetime cumulative risk of about 2%. The condition primarily affects patients between 20 and 50 years old, with a peak frequency observed between 35 and 50 years.After menopause, due to the natural involution of the gland, Bartholin's cysts and abscesses become less frequent, although they can still occur. Moreover, in women over 50, the appearance of a new mass in the gland region should prompt caution, as it may, in rare cases, indicate a carcinoma of the Bartholin's gland or an adjacent vulvar cancer. Therefore, for patients over 40 presenting with a newly emerged cyst or abscess, clinical guidelines recommend performing a biopsy or excision to rule out malignancy. We present the case of a 50-year-old woman with no significant medical history, who was urgently referred to the gynecological emergency department due to confusion, unexplained fever of 40 °C, and resistant leucorrhoea following a week of corticosteroid antibiotic therapy. Clinical examination revealed a large, tender right vulvar mass, indicative of an acute Bartholin's abscess. The patient exhibited signs of septic shock and was admitted to the ICU. Following a diagnosis of sepsis, broad-spectrum antibiotic therapy was initiated, alongside fluid resuscitation and norepinephrine support. Surgical drainage of the abscess confirmed the presence of E. coli. The patient's condition improved rapidly, and she was discharged on postoperative day 8 with no complications. This case underscores that while Bartholin's abscess is typically benign, severe complications, including septic shock, can occur—especially in patients over 50. The appearance of a new Bartholin's region mass in older women should prompt consideration of malignancy, necessitating biopsy or excision. Recent studies compare various therapeutic approaches including simple incision and drainage, Word catheter placement, marsupialization, silver nitrate application, and complete gland excision. Each method has its advantages and drawbacks, with marsupialization offering lower recurrence rates and higher patient satisfaction in many instances.
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