Τρίτη 14 Αυγούστου 2018

Extensive abdominal wall ulceration as a late manifestation of antiphospholipid syndrome: a case report

Antiphospholipid syndrome is an autoimmune disorder characterized by the presence of antiphospholipid antibodies and commonly presents with vascular thromboembolic phenomena, thrombocytopenia, and obstetric co...

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Unilateral pulmonary edema: a case report and review of the literature

Unilateral pulmonary edema is an uncommon condition and is a rare clinical entity that is often misdiagnosed at the initial stages. In a majority of patients it occurs in the upper lobe of the right lung. Ther...

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Adenocarcinoma within a tailgut cyst

Description 

We present a case of a 53-year-old woman with painful defaecation and lower abdominal pain. Her medical history and laboratory testing were unremarkable. An abdominopelvic CT scan was performed. Axial non-enhanced CT image (figure 1) and postcontrast CT image (figure 2) revealed a well-defined, slightly lobular soft tissue density mass in the retrorectal/presacral space. It shows a thin peripheral calcification and internal enhancing components after administration of intravenous contrast material. These features suggest a rare developmental retrorectal lesion, with enteric cysts (tailgut cyst and cystic rectal duplication) being the more common. Differential diagnosis comprises lymphangioma, epidermoid cyst, dermoid cyst, endometrioma, rectal duplication cyst, anal gland cyst and anterior meningocoele.

Figure 1

Axial non-enhanced CT image showing a heterogeneous nodular mass of tissue density with a tiny peripheral calcification in the presacral space.

Figure 2

Axial and sagittal postcontrast...



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Gallbladder empyema complicating acute myeloid leukaemia in an adolescent boy

Gallbladder (GB) empyema is an uncommon complication in acute myeloid leukaemia (AML). Non-specific signs and symptoms and rarity of disease in AML make it difficult to diagnose early. We report a case of 13-year-old boy who had AML and developed GB empyema in the neutropaenic period. The patient was managed with antibiotics, ultrasound-guided tapping and interval cholecystectomy and recovered well. GB empyema is a life-threatening complication which should be kept as a possibility while evaluating patients with neutropaenia with pain abdomen localised to right hypochondrium as early diagnosis and treatment can lead to better outcomes.



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Thyroid metastasis from rectal adenocarcinoma

The thyroid is a rare site for metastasis, occurring in 0.1% of colorectal cases. A 46-year-old man with rectal adenocarcinoma developed an enlarging anterior neck mass and increasing carcinoembryonic antigen 2 years after curative intent treatment. Imaging showed aggressive features with invasion of the larynx, trachea and oesophagus, suspicious for anaplastic thyroid carcinoma. The patient underwent tumour debulking with neck dissection and tracheostomy. Final histopathological review revealed metastatic adenocarcinoma from a colorectal primary. Despite chemotherapy, the patient eventually succumbed to disease progression and complications of his illness. This case demonstrates that recognising rare sites of metastasis may help clinicians effectively institute earlier intervention.



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Aneurysmal fibrous histiocytoma

Aneurysmal fibrous histiocytoma (AFH) is a type of fibrohistiocytic tumour. We present a case of a patient who presented with a skin papule on the thigh region. Histopathological examination following total excision of the lesion revealed an AFH. No sign of recurrence was present 6 months after surgery.



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Prostatitis, a rare presentation of granulomatosis with polyangiitis, successfully treated with rituximab and prednisone

A previously healthy 21-year-old man presented with an 8-month history of weight loss, lethargy and dysuria unresponsive to empiric antibiotics and paraurethral drainage of a prostatic abscess. Urinalysis showed pyuria, but cultures failed to grow any organisms. Additionally, he developed new onset sensorineural hearing loss. CT of the chest showed two right-sided cavitary lesions. CT of the abdomen and pelvis demonstrated a prostatic abscess. A prostate biopsy demonstrated necrotising granulomatous prostatitis. A lung biopsy showed necrotising granulomatous inflammation. He was diagnosed with granulomatosis with polyangiitis (GPA). He was successfully treated with rituximab and prednisone. At 6-month follow-up, he continued to be in remission with resolution of his symptoms. This case demonstrates a rare presentation of prostatitis as the presenting symptom of GPA. As far as we know, this case is the first documented report of rituximab and prednisone as successful therapy for prostatitis secondary to GPA.



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Epstein-Barr-positive classical Hodgkin lymphoma-associated haemophagocytic lymphohistocytiosis: a rare case

Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition, which is usually triggered by autoimmune disorders, viral infections and malignancy, like lymphomas. We present a 60-year-old Hispanic woman with a medical history of hypertension and systemic lupus erythematosus presenting with fever, generalised weakness and shortness of breath for 3 weeks. She was hypotensive on presentation, and a CT scan of abdomen/chest showed multiple irregular hepatic and splenic hypodense lesions. A liver biopsy revealed Classical Hodgkin lymphoma (cHL) with positive Epstein-Barr virus (EBV) staining that was later confirmed with high serum EBV DNA levels. Incidentally, a liver biopsy disclosed haemophagocytosis in some cells. HLH-associated cHL is an uncommon condition that can cause severe systemic symptoms acting as the perfect mimic of septic shock, deviating the clinical eye toward treating with antibiotics and not addressing in a timely manner the real aetiology of the patient's condition.



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Severe thromboembolic phenomenon in the setting of pseudoprogression of melanoma brain metastases in response to combination immunotherapy

Approximately half of patients with metastatic melanoma develop brain metastasis (MBM) in their disease course. However, patients with MBM were often excluded from early immunotherapy trials, and therefore, the role of immunotherapy in these patients is less clear. We review the case of a patient with widespread metastatic melanoma and symptomatic brain metastases at initial diagnosis. In this case, we have demonstrated that it is reasonable to pursue combination ipilimumab and nivolumab in borderline performance status patients with extensive brain metastases. Additionally, this case teaches us to be vigilant for severe autoimmune toxicities such as severe thrombotic events in the setting of pseudoprogression of brain metastases. We discuss this case in the context of the current melanoma literature.



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Post-traumatic aortic pseudoaneurysm causing acquired pulmonary artery stenosis

Description 

A 43-year-old man with a diagnosis of severe pulmonary stenosis was referred to us for pulmonary valve balloon dilatation. We reviewed the case and found that the patient was complaining of progressively increasing dyspnoea over the past one year. He did not complain of chest pain, syncope, limb swelling or cyanosis. History was unremarkable except for a motorbike accident which he suffered 10 years back and sustained injuries over his face, chest and limbs. He made full functional recovery within 2 months after the accident and remained free from any symptoms for the next 9 years.

We reviewed his echocardiogram and found that he had dilated right atrium and right ventricle. Left-sided chambers and valves were normal. The pulmonary valve was normal. Instead, he had supravalvular pulmonary stenosis caused by compression of the pulmonary artery by an extrinsic mass (figures 1 and 2). CT...



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Penile fracture after priapism due to sildenafil ingestion: out of frying pan into the fire

Description  

A 36-year-old man presented to our department with complaints of swelling, pain and deformed penis for 2 days. He had a history of 100 mg sildenafil ingestion, following which he sustained a prolonged erection that persisted even after sexual intercourse. He tried vigorous masturbation to relieve it, but he failed. After this exercise, while he was sleeping, his child mistakenly fell over his erect penis, following which he developed sudden severe pain in his penis and lost tumescence. On examination, he had swelling and ecchymosis on the right posterolateral aspect of his penis (figure 1). There was no haematuria or blood at meatus. On the basis of history and physical examination, a diagnosis of penile fracture was made. Ultrasound revealed a tear in the lateral wall of the right corpora cavernosa with a large haematoma. The patient was taken for emergency exploration. Intraoperatively, a defect of approximately 1x1 cm was visualised...



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Aphallia: do sociocultural issues affect treatment decisions?

Description 

Otherwise healthy 1-month-old male patient (documented 46,XY karyotype) was referred to the plastic surgery unit for assessment. Physical exam revealed absence of penis and urethra along with a well-formed scrotum and both normally descended testicles (figure 1). No characteristic facial phenotype was noted. Also, there were no abdominal wall hernias nor abnormalities of the anal canal. An echocardiogram, abdominal ultrasound and CT were performed to exclude other malformations. CT scan revealed a functional but hypoplastic right kidney and the presence of a vesicorectal fistula. The rest of the studies including a hormonal profile were within normal limits.

Figure 1

Picture showing the perineal region of the patient.

Surgical fistula closure and perineal urethrostomy placement were recommended as initial treatment. Also, the possibility of either early female sexual reassignment or phalloplasty were proposed as long-term options for definitive reconstruction. The...



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Bilateral primary adrenal lymphoma successfully treated with non-CHOP chemotherapy regimen

Primary bilateral non-Hodgkin's lymphoma of the adrenal gland is a rare presentation with <200 reported cases. We report a 70-year-old man who presented with vomiting and abdominal pain for 2 weeks and was subsequently diagnosed to have high-grade lymphoma of the adrenal glands. The patient was then started on a rituximab–bendamustine chemotherapy regimen. The patient tolerated treatment well without significant side effects. Repeat positron emission tomography CT scan after four cycles of chemotherapy showed complete response. The patient has completed six cycles of chemotherapy and is on regular follow-up with no signs of relapse.



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