Three synchronous primary carcinomas in a patient with HNPCC associated with a novel germline mutation in MLH1: Case report

Background

Adnexal masses are not uncommon in patients with breast cancer. Breast cancer and ovarian malignancies are known to be associated. In patients with breast cancer and co-existing pleural effusions, ascites and adnexal masses, the probability of disseminated disease is high. Nevertheless, benign ovarian masses can mimic this clinical picture when they are associated with Meigs' syndrome making the work-up and management of these patients challenging. To our knowledge, there are no similar reports in the literature and therefore we present this case to highlight this entity.

Case presentation

A 56-year old woman presented with a 4 cm, grade 2, invasive ductal carcinoma of her left breast. Pre-treatment staging investigations showed a 13.5 cm mass in her left ovary, a small amount of ascites and a large right pleural effusion. Serum tumour markers showed a raised CA125 supporting the malignant nature of the ovarian mass. The cytology from the pleural effusion was indeterminate but thoracoscopic biopsy failed to show malignancy. The patient was strongly against mastectomy and she was commenced on neo-adjuvant Letrozole 2.5 mg daily with a view to perform breast conserving surgery. After a good response to the hormone manipulation, the patient had breast conserving surgery, axillary sampling and laparoscopic excision of the ovarian mass which was eventually found to be a benign ovarian fibroma.

Conclusion

Despite the high probability of disseminated malignancy when an ovarian mass associated with ascites if found in a patient with a breast cancer and pleural effusion, clinicians should be aware about rare benign syndromes, like Meigs', which may mimic a similar picture and mislead the diagnosis and management plan.     

Tracheal adenoid cystic carcinoma masquerading asthma: A case report

Background

Hemorrhagic pleural effusion, especially in the right hemithorax rarely occurs as the sole presentation of pancreatitis.

Case Presentation

This article reports massive right-sided hemorrhagic pleural effusion as the sole manifestation of pancreatitis in a 16-year-old Iranian boy. The patient referred to Nemazee Hospital, the main hospital of southern Iran, with right-sided shoulder and chest pain accompanied with dyspnea. His chest x-ray showed massive right-sided pleural effusion. The pleural fluid amylase was markedly elevated (8840 U/L), higher than that in the serum (3318 U/L). Abdominal CT scan showed a cystic structure measuring about 5·2 cm in the head of pancreas, highly suggestive of a pancreatic pseudocyst. Pleural effusion resolved after 3 weeks of chest tube insertion but not completely. After this period of conservative therapy another CT scan showed that pseudocyst was still in the head of pancreas. So, external drainage was done with mushroom insertion and the patient was discharged after 40 days of hospitalization. The cause of pancreatitis could not be identified.

Conclusion

Pancreatitis should be taken into consideration when hemorrhagic pleural effusion, especially in the right hemithorax occurs.     

Perioperative Challenges and Surgical Treatment of Large Simple,and Infectious Liver Cyst - A 12-Year Experience

Background

Cystic lesions of the liver consist of a heterogeneous group of disorders that can present diagnostic and therapeutic challenges.

Methods

A retrospective review of all medical records of adult patients diagnosed with large (>7 cm) cystic lesions of the liver between January 2000 and December 2011, at Kurume University Hospital. Cases with polycystic disease were excluded.

Results

Twenty three patients were identified. The mean size was 13.9 cm (range, 7-22cm). The majority of simple cysts were found in women (females: males, 2: 21). In 19 patients, the cyst was removed surgically by wide deroofing (laparoscopically in 16 cases, combined with ethanol sclerotherapy in 13 cases). Infection of the liver cyst occurred in one patient, who later underwent central bi-segmentectomy.

Conclusion

Simple large cysts of the liver can be successfully treated by laparoscopic deroofing and alcohol sclerotherapy. Large hepatic cyst considered to need drainage should be removed surgically to avoid possible infection.     

Predicting invasion in mammographically detected microcalcifcation: a preliminary report

Background

Malignant pleural mesothelioma is a rare malignancy. The outcome remains poor despite complete surgical resection.

Patients and methods

Eleven patients with histologicaly proven epithelial type malignant pleural mesothelioma undergoing extrapleural pneumonectomy with systemic chemotherapy and/or radiotherapy before and after surgical resection were retrospectively reviewed.

Results

Ten out of 11 patients underwent complete surgical resection, of these 7 patients had stage I disease. Of these 7 patients, 5 are alive without any recurrence, a 2-year survival rate of 80% was observed in this group. There was no operative mortality or morbidity.

Conclusion

Extrapleural pneumonectomy with perioperative adjuvant treatment is safe and effective procedure for epithelial type malignant pleural mesothelioma.     

Primary pleural leiomyosarcoma with rapid progression and fatal outcome: a case report

Introduction

Leiomyosarcomas are neoplasms of smooth muscles that most commonly arise from the uterus, gastrointestinal tract, or soft tissue. Primary pleural leiomyosarcoma is extremely rare. To the best of our knowledge, only nine cases have been published to date. Because of the rarity of pleural leiomyosarcoma and its similarity (clinical and histological) to other pleural neoplasms, particularly sarcomatous mesothelioma, diagnosis is often difficult.

Case presentation

A 58-year-old North African man was admitted with complaints of dyspnea and chest pain to our hospital. Chest computed tomography revealed right pleural effusion and pleural thickening. A transthoracic needle biopsy yielded a diagnosis of leiomyosarcoma, and tumor cells were strongly and uniformly positive for vimentin, a smooth muscle actin at immunohistochemical analysis. A general examination did not show any metastatic lesions in other areas. One month after diagnosis, the tumor grew rapidly, with pulmonary invasion, and therefore he was treated only by palliative care. He died from respiratory failure one month later. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma.

Conclusions

Although leiomyosarcoma originating from the pleura is rare, this entity is increasingly described. The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a pleural mass is identified.     

Assessment of a bedside test for N-terminal pro B-type natriuretic peptide (NT-proBNP) to differentiate cardiac from non-cardiac causes of pleural effusion in cats

Background

Cats with pleural effusion represent common emergencies in small animal practice. The aim of this prospective study was to investigate the diagnostic ability of a point-of-care ELISA (POC-ELISA) for the measurement of N-terminal pro B-type natriuretic peptide (NT-proBNP) to differentiate cardiac from non-cardiac disease in cats with pleural effusion. The sample material for use of this rapid test was either plasma or diluted pleural effusion.Twenty cats with moderate to severe pleural effusion were prospectively recruited. The cats were grouped into two groups, with or without congestive heart failure (CHF; N-CHF), after complete work-up. Blood and effusion were collected in EDTA tubes. Plasma and pleural effusion supernatants were transferred into stabilizer tubes and frozen. POC-ELISA for NT-proBNP was performed with plasma and diluted effusion (1:1). Quantitative NT-proBNP measurement was performed in plasma and diluted and undiluted effusions.

Results

Six cats were assigned to the CHF group. Of the 14 cats in the N-CHF group, 6 had concurrent cardiac abnormalities that were not responsible for the effusion. For the detection of CHF, the test displayed respective sensitivities and specificities of 100% and 79% in plasma and 100% and 86% in diluted pleural fluid. Receiver operating characteristic (ROC) analysis for quantitative NT-proBNP measurement of plasma and diluted and undiluted pleural effusions displayed areas under the curve of 0.98, sensitivities of 100% and specificities of 86%. The optimum cut-off was calculated at 399 pmol/l in plasma and 229 pmol/l in the diluted effusion and 467 pmol/l in the undiluted effusion.

Conclusions

POC-ELISA for NT-proBNP in both plasma and diluted pleural effusion was suitable to differentiate cardiac from non-cardiac causes of feline pleural effusion. According to our results, use of pleural effusion is feasible, but dilution of the effusion before measurement seems to improve specificity.

     

Results of chemical pleurodesis with mitoxantrone in malignant pleural effusion from breast cancer

Background

Carcinoma of the breast is the second leading cause of malignant pleural effusions. This study reports on the efficacy of mitoxantrone as a sclerosing agent in patients with breast cancer who had a pleural effusion as a direct consequence of metastatic disease.

Patients and methods

Over a 5-year period, 114 patients with a known breast malignancy and having recurrent symptomatic pleural effusion referred for chest tube drainage and sclerotherapy were considered eligible. They had received no prior intrapleural therapy and had a predicted survival of >1 month. All of them underwent pleural drainage and chemical pleurodesis with mitoxantrone. Survival, complications and response to pleurodesis according to clinical and radiographic criteria were recorded. The data are expressed as the mean ± standard error of the mean (SEM) and the median. The χ² test was used for statistical analysis. To assess the prognostic value of Karnofsky's performance status score a Cox proportional hazards model was used.

Results

The mean age of the patients was 53.5 ± 2.1 years. Effusion occurred after 38.2 ± 6.2 months (range: 1–229 months) after the diagnosis. Ipsilateral effusion was seen in 73%, contralateral in 20% and bilateral in 7%. Forty patients (35%) had pleural effusion as the first evidence of recurrence. The mean volume of effusion drained was 1020 ± 125 ml and the chest tube was removed within 5 days in 82% of patients. Side effects of chemical pleurodesis included mainly fever, chest pain, nausea and vomiting. At 30 days 64 patients (56.3%) had a complete response (CR) and 30 patients (26.3%) partial response (PR) to pleurodesis (overall response: 82.6%). At 60 days the overall response was 78.5% (CR:53.5%, PR: 25%). The mean survival was 15.6 ± 2 months. Karnofsky's performance status score was found to be a statistically significant predictor. Patients with Karnofsky's performance status score >70 had a median survival of 513 days, as opposed to a median survival of only 63 days for patients with a Karnofsky's performance status score <30.

Conclusions

Mitoxantrone is effective in the treatment of malignant pleural effusion due to breast carcinoma with relatively low local or systemic toxicity. Karnofsky's performance status score at the time of pleurodesis is predictive of survival.

     

Une syringocèle révélée par un abcès périntéal

Purpose

The authors present a clinical report of a perforate syringocele diagnosed in a male patient and they review the clinical, radiological and therapeutic aspects of this disease.

Case report

A 50-year-old man attended the emergency room with perineal abscess. Computerized Tomography (CT) scan was used to establish the diagnosis. This patient also underwent urethrocystoscopy, sonography and retrograde urethrography.

Results

Treatment consisted of open surgical evacuation of the perineal abscess with bladder catheter. Urethrocystoscopy showed a large perforate syringocele in the urethra with no need for endoscopy marsupialization. No recurrence was observed at follow-up at 3 months.

Conclusion

Syringocele is a rare malformation which usually is asymptomatic. It can be congenital or acquired and can be classified into four morphological types. The most frequent treatment is endoscopic marsupialization of the syringocèle. Open surgical excision is recommended for complicated syringocèle with abscess or perineal pain.     

Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case report

Background

Liposarcomas are among the most common sarcomas of adult life. Pleomorphic liposarcoma, characterized by pleomorphic lipoblasts, is the rarest subtype. To our knowledge only three cases of pleomorphic liposarcoma of the foot or ankle have been reported so far.

Case presentation

A 71-year-old female presented with a large growing mass on the dorsum of her right foot. Computed tomography showed invasive tumorous mass. Excision biopsy revealed the mass to be a pleomorphic liposarcoma, and below the knee amputation was performed.

Conclusion

Although the incidence of pleomorphic liposarcoma in the foot is very low, it is essential to perform thorough histological analysis of all soft tissue masses, regardless of their benign appearance, because only prompt radical surgery can result in a good prognosis for the patient.     

"Floating arm" injury in a child with fractures of the proximal and distal parts of the humerus: a case report

Introduction

Primary malignant pericardial mesothelioma is a very rare pericardial tumor of unknown etiology.

Case presentation

A 61-year-old Caucasian woman was admitted to our hospital complaining of exertional dyspnea due to a large pericardial effusion. Intrapericardial fluid volume declined after repeated pericardiocentesis, but the patient progressively developed a hemodynamically relevant pericardial constriction. Pericardiectomy revealed a pericardial mesothelioma. Subsequently, four cycles of chemotherapy (dosage according to recently published trials) were administered. The patient remained asymptomatic, and there was no recurrence of the tumor after three years.

Conclusion

Pericardial mesothelioma should be considered and managed appropriately in non-responders to pericardiocentesis, and in patients who develop constrictive pericarditis late in their clinical course.     

Etanercept in the treatment of recalcitrant enteropathic arthritis: a case report

Introduction

Enteropathic arthritis is one of the recognized extraintestinal manifestations of inflammatory bowel disease and affects up to 25% of patients. The treatment options for refractory disease were rather limited and ineffective until the arrival of biologic therapy in the last few years. The use of etanercept was unique for this disease.

Case presentation

In this case report, a 58-year-old Malay woman with a 17-year history of ulcerative colitis had persistent left knee effusion and synovitis for seven years, despite remission of the primary disease. She had had multiple courses of systemic and intra-articular steroid that caused significant systemic side effects such as impaired fasting glucose, hypertension, cataract, and weight gain. She also had a total left knee replacement for secondary osteoarthritis. But the left knee synovitis and effusion recurred a month after the total knee replacement, and she was subjected to a total synovectomy the following year. In view of failure of remission despite multiple immunosuppressants (100 mg of azathioprine daily, 1 g of sulfasalazine twice a day, 10 mg of prednisolone daily, and 10 mg of methotrexate weekly), 25 mg of subcutaneous etanercept twice weekly was started. After 5 weeks of treatment, complete resolution of left knee effusion and normalization of the inflammatory markers were shown. This continued up to 12 months of follow-up while our patient was on etanercept and 10 mg of methotrexate weekly. No relapse or serious side effects were noted.

Conclusions

This case demonstrates the efficacy of etanercept in recalcitrant enteropathic arthritis with no relapse of the underlying colitis while on treatment. The usage of this tumor necrosis factor inhibitor was unique in this case of rheumatology and gastroenterology.     

Primary Kaposi sarcoma of the subcutaneous tissue

Background

Tumor recurrence following radical cystectomy for a low-grade superficial transitional cell carcinoma (TCC) is exceedingly uncommon and has not been reported previously.

Case presentation

We describe a case of a young male presenting with anorexia, weight loss and a large, painful locally destructive pelvic recurrence, ten years after radical cystoprostatectomy. The pathology was consistent with a low-grade urothelial carcinoma. After an unsuccessful treatment with cisplatin-based chemotherapy, the patient underwent a curative intent hemipelvectomy with complete excision of tumor and is disease free at one year follow-up.

Conclusion

A literature review related to this unusual presentation is reported and a surgical solutions over chemotherapy and radiotherapy is proposed.     

Lung in Dengue: Computed Tomography Findings

Background

Dengue is the most important mosquito-borne viral disease in the world. Dengue virus infection may be asymptomatic or lead to undifferentiated fever, dengue fever with or without warning signs, or severe dengue. Lower respiratory symptoms are unusual and lung-imaging data in patients with dengue are scarce.

Methodology/Principal Findings

To evaluate lung changes associated with dengue infection, we retrospectively analyzed 2,020 confirmed cases of dengue. Twenty-nine of these patients (11 females and 18 males aged 16–90 years) underwent chest computed tomography (CT), which yielded abnormal findings in 17 patients: 16 patients had pleural effusion (the sole finding in six patients) and 11 patients had pulmonary abnormalities. Lung parenchyma involvement ranged from subtle to moderate unilateral and bilateral abnormalities. The most common finding was ground-glass opacity in eight patients, followed by consolidation in six patients. Less common findings were airspace nodules (two patients), interlobular septal thickening (two patients), and peribronchovascular interstitial thickening (one patient). Lung histopathological findings in four fatal cases showed thickening of the alveolar septa, hemorrhage, and interstitial edema.

Conclusions/Significance

In this largest series involving the use of chest CT to evaluate lung involvement in patients with dengue, CT findings of lower respiratory tract involvement were uncommon. When abnormalities were present, pleural effusion was the most frequent finding and lung involvement was often mild or moderate and bilateral. Extensive lung abnormalities are infrequent even in severe disease and when present should lead physicians to consider other diagnostic possibilities.     

Detection of somatostatin receptors in human osteosarcoma

Background

Morbid obesity strongly predicts morbidity and mortality in surgical patients. However, obesity's impact on outcome after major liver resection is unknown.

Case presentation

We describe the management of a large hepatocellular carcinoma in a morbidly obese patient (body mass index >50 kg/m²). Additionally, we propose a strategy for reducing postoperative complications and improving outcome after major liver resection.

Conclusion

To our knowledge, this is the first report of major liver resection in a morbidly obese patient with hepatocellular carcinoma. The approach we used could make this operation nearly as safe in obese patients as it is in their normal-weight counterparts.     

Evaluation of SHOX copy number variations in patients with Müllerian aplasia

Background

Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment.

Methods

From January 2000 all patients under 18 years of age diagnosed with "rare paediatric tumours" were centrally registered by the Italian centres participating in the TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study.

Results

Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb. All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure. Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease.

Conclusions

Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment.     

Evaluation of ultrasound lung comets by hand-held echocardiography

Background

Most patients with hypertrophic cardiomyopathy (HCM) have asymmetric septal hypertrophy and among them, 25% present dynamic subaortic obstruction. Apical HCM is unusual and mid-ventricular HCM is the most infrequent presentation, but both variants may be associated to an apical aneurysm. An even more rare presentation is the coexistece mid-ventricular and apical HCM. This case is a combination of obstructive HCM with mid-ventricular HCM and an apical aneurysm, which to date, has not been reported in the literature.

Case presentation

The patient is a 49 year-old lady who presents a combination of septal asymmetric hypertrophic cardiomyopathy (HCM) and midventricular HCM, a subaortic gradient of 65 mm Hg and a midventricular gradient of 20 mm Hg, plus an apical aneurysm. Her clinical presentation was an acute myocardial infarction in June 2005. One month after hospital discharge, the electrocardiogram (ECG) showed a right bundle branch block (RBBB) with no Q waves or ST segment elevation. Coronary angiography revealed normal coronary arteries, left ventricular hypertrophy and an apical aneurysm.

Conclusion

This case is a rare example of an asymptomatic patient with subaortic and mid-ventricular hypertrophic cardiomyopathy, who presents a myocardial infarction and normal coronary arteries, and during the course of her disease develops an apical aneurysm.     

Ring chromosome 13 syndrome characterized by high resolution array based comparative genomic hybridization in patient with 47, XYY syndrome: a case report

Introduction

Percutaneous or endocavitory drainage of a diverticular abscess under radiological guidance often enables one to perform a one-staged resection and anastomosis (without stoma formation) instead of a two-staged procedure. It reduces the significant postoperative morbidity and mortality associated with the conventional emergency surgical management. However, radiological guidance is not always available due to limited resources during out-of-hours.

Case presentation

A 78-year-old Caucasian woman underwent transrectal drainage of a diverticular abscess performed with a pigtail catheter without radiological guidance. Technical details of the procedure are described and alternative options discussed.

Conclusion

In carefully selected patients, per-rectal drainage using a pigtail catheter can be performed without radiological guidance and the procedure offers a simple and effective way of controlling sepsis.     

Prostate embryonal rhabdomyosarcoma in adults: Case report and review of literature

Introduction

Prostate embryonal rhabdomyosarcoma (ERMS) is a common tumour in infants and children, with a median occurrence age of 5 years, but it is rare in adults. It is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethral obstruction, and systemic spread, commonly to the lungs, liver and bone. Several therapeutic approaches have been employed in the effort to treat prostate ERMS, but all of them have failed to gain a significant survival benefit in adult patients.

Case report

We report on a case of a stage IV prostate ERMS, approached with combined-modality treatment, with the administration of 5 courses of doxorubicin, ifosfamide and 2-mercaptoethane sulfonate sodium (mesna), and, subsequent radiotherapy to the prostatic bed (60 Gy/30 fxs). The patient remained free of progression of disease for about 1 year to finally experience a systemic relapse with multiple lung metastases and pleural effusion. The patient died for metastatic disease 27 months following the initial diagnosis.

Conclusion

While it remains questionable which therapeutic approach for prostate ERMS in adults is the most appropriate, our report demonstrates that a chemo-radiation combined treatment can control the prostate disease, reducing the symptoms and improving the quality of life of these patients, for the most part destined to die for systemic progression of disease.     

Therapeutic activity of two xanthones in a xenograft murine model of human chronic lymphocytic leukemia

Background

A 24-year-old female patient was diagnosed with classic Hodgkin's lymphoma in clinical stage II, and combination chemotherapy followed by radiotherapy was initiated. During the following 5 years, the disease progressed despite several standard therapeutic approaches, including autologous and allogeneic stem cell transplantation.

Methods

Lenalidomide (25 mg daily) treatment was then initiated in a continuous dosing schedule. Positron emission tomography scans were performed before and during lenalidomide treatment. Hematologic and laboratory values, as well as physical condition were also assessed before and during lenalidomide treatment.

Results

Four months after continuous lenalidomide treatment, tumor load was significantly reduced, B symptoms had resolved, and the patient's physical condition had improved, allowing her to resume normal daily-living activities. Evaluations after 15 months of lenalidomide treatment indicated limited disease progression. Nevertheless, the patient was feeling well and maintaining a normal active life. Treatment was well tolerated, allowing the patient to remain on continuous dosing, which has now been maintained for 18 months.

Conclusion

Daily, long-term lenalidomide treatment provided clinical benefit and was well tolerated in a patient with relapsed, advanced classic Hodgkin's lymphoma.