Πέμπτη 1 Νοεμβρίου 2018

Adult testicular granulosa cell tumour: an extremely rare entity

Adult granulosa cell tumours are extremely rare and usually benign but sometimes can assume an aggressive behaviour. A 31-year-old man presented with a 45 mm testicular mass. Radical orchiectomy was performed. Histological examination showed elongated cells, with hyperchromatic nuclei and Call-Exner bodies. Immunohistochemical analysis revealed positivity to vimentin, inhibin and negative PLAP and AE1/AE3 staining. The patient did not receive any adjuvant therapy and remained asymptomatic during 10 years. There are few cases of adult granulosa cell tumours and there is no consensus regarding malignancy criteria, treatment and follow-up. The description of these entities is crucial to allow a better management of these patients.



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Acute myeloid leukaemia relapse presenting as cardiac myeloid sarcoma

A 50-year-old woman previously diagnosed with acute myeloid leukaemia presented with a 3-month history of shortness of breath and a right-sided facial rash. A chest CT revealed an intracardiac mass in the right atrium extending into her superior and inferior vena cava. Surgery was performed to remove the mass and pathology was consistent with myeloid sarcoma. After surgery, adjuvant radiation therapy was directed to the residual disease. The patient eventually relapsed in other sites not including the right atrium and eventually succumbed to her disease.



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Incidental discovery of Amyands hernia

Description 

A 58-year-old woman was referred to Hospital Tuanku Ja'afar (Seremban, Malaysia) for contrast-enhanced CT abdomen and pelvis following discovery of a solid mass in the right adnexa on ultrasonography. CT findings revealed a well-defined, fat-containing mass with calcifications in the right adnexa measuring 4.5x7.8x4 cm, consistent with ovarian teratoma (figure 1). Incidentally, the vermiform appendix appeared to be elongated and extended into the right inguinal canal. It was not enlarged, and there was no surrounding fat streakiness (figures 1 and 2). A diagnosis of Amyand's hernia was made. She was advised for conservative (non-surgical) treatment as she was asymptomatic for both ovarian mass and Amyand's hernia. She was then given appointment for ultrasound follow-up.

Figure 1

Contrast-enhanced CT abdomen and pelvis in axial sections showing the appendix (arrows) extending into the right inguinal canal. Right ovarian teratoma is also visualised (asterisk).



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Congenital colonic stenosis with absent caecum and appendix: a rare association

Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung's disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.



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Central serous chorioretinopathy in a patient of juxtapapillary excavation misdiagnosed as optic disc pit maculopathy

A 29-year-old healthy man had blurring of vision in the left eye for the past 2 months and was referred for the surgical management of optic disc pit maculopathy. Colour fundus examination and optical coherence tomography (OCT) revealed a large deep retinochoroidal excavation close to the temporal edge of the optic disc with an isolated central neurosensory detachment at the macula with underlying multiple small pigment epithelium detachments in the absence of retinoschisis. Fundus fluorescein angiography (FFA) confirmed the presence of multi-focal leakage at the macula and pooling into subretinal space in the form of a 'smoke-stack'. A correct diagnosis of juxtapapillary excavation and central serous chorioretinopathy was made and lifestyle modifications were advised in view of the acute episode. The clinical signs, OCT and FFA feature helped in the differentiation and appropriate management of maculopathy in this case.



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Multifocal avascular osteonecrosis despite appropriate anticoagulation therapy in a patient with systemic lupus erythematosus and antiphospholipid syndrome

Multifocal avascular osteonecrosis (AON) is a serious manifestation of systemic lupus erythematosus (SLE). Prothrombotic factors, especially antiphospholipid antibodies (aPL), have been associated with the development of AON; therefore, attenuating the procoagulant state while balancing the haemorrhagic risks might have a rationale when managing this condition. We report a case of a 37-year-old patient with SLE, treated with low doses of corticosteroids and immunosuppressive therapy, who was started on vitamin K antagonist following an episode of deep vein thrombosis while having persistent positivity for aPL. After 2 years, he presented with multifocal AON, involving both femurs and shoulders. The patient underwent a bilateral hip replacement, but despite appropriate anticoagulation therapy after 2 years, he developed another episode of AON at both distal epiphyses of the femurs and proximal epiphyses of the tibias. Multifocal AON should be suspected, especially in the presence of aPL positivity. Its aetiology is still unknown and is most likely multifactorial. Its management is challenging and requires combined approaches.



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Challenges facing a woman wishing to report intimate partner violence in Belize

We present a case of a 29-year-old woman who presented to a volunteer-run primary care facility in Southern Belize. Her initial presentation was vaginal itching and white discharge; she also requested insertion of a sub-dermal contraceptive implant. During the insertion, marks suspicious for deliberate self-harm were noticed on the patient's arm, and on further exploration she revealed she was being physically and emotionally abused by her husband. With some encouragement, she requested help in taking further action to preserve her safety; however, in Belize clinicians have no power to assist in cases involving adults. Therefore, the victim should self-present to a police station, resulting in a significant potential barrier to reporting intimate partner violence (IPV). Here we discuss this barrier further, as well as other barriers that exist to reporting IPV, and discuss possible policy changes that may improve the situation in Belize.



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