Primary soft tissue Ewing’s sarcoma of the maxillary sinus in elderly patients: presentation,management and prognosis

Nonosseous or soft tissue Ewing’s sarcoma is a rare form of Ewing’s sarcoma/primitive neuroectodermal tumour that seldom affects the head and neck region. Involvement of the nose and paranasal sinuses is extremely uncommon, with only eight of such patients being reported to date, mostly affecting adolescents and young adults. To our knowledge, this study is the first comprehensive report of primary soft tissue Ewing’s sarcoma involving the paranasal sinuses in an elderly patient who successfully completed treatment. We herein discuss the pathogenesis, management and factors affecting the prognosis of this rare group of tumours involving the nose and paranasal sinuses, in relation to the available literature.     

Neurogenic heterotopic ossification after a stroke: diagnostic and radiological challenges

Heterotopic ossification (HO) is the aberrant formation of ectopic bone within the soft tissues, of which the aetiology is usually either traumatic or neurogenic. Neurogenic HO is a known but uncommon complication that occurs after a cerebral or spinal insult. The condition may present with a spectrum of symptoms and is often difficult to diagnose clinically. Although different imaging modalities have been used to diagnose HO, clinicians and radiologists may occasionally encounter radiological features of HO that may mimic other disease conditions. We herein report a rare case of neurogenic HO occurring in the non-paretic limb of a patient, and the diagnostic and radiological challenges encountered.     

Intracavernous internal carotid artery pseudoaneurysm

Epistaxis is commonly encountered in otorhinolaryngologic practice. However, severe and recurrent epistaxis is rarely seen, especially that originating from a pseudoaneurysm of the intracavernous internal carotid artery (ICA). We herein present the case of a 32-year-old man who was involved in a motor vehicle accident and subsequently developed recurrent episodes of profuse epistaxis for the next three months, which required blood transfusion and nasal packing to control the bleeding. Computed tomography angiography revealed a large intracavernous ICA pseudoaneurysm measuring 1.7 cm × 1.2 cm × 1.0 cm. The patient underwent emergent four-vessel angiography and coil embolisation and was discharged one week later without any episode of bleeding. He remained aasymptomatic after three-month and one-year intervals. This case report highlights a large intracavernous ICA pseudoaneurysm as a rare cause of epistaxis, which requires a high index of suspicion in the right clinical setting and emergent endovascular treatment to prevent mortality.     

Imaging features of an intraosseous arteriovenous malformation in the tibia

Primary intraosseous arteriovenous malformations (AVMs) are rare and have only been occasionally reported. We herein report a histologically proven case of primary intraosseous AVM in the tibia, which mimicked a fibrous tumour on radiography. This presentation carries a risk of triggering acute large haemorrhage through unnecessary biopsy. In intraosseous AVM, the magnetic resonance (MR) imaging features typical of a soft tissue AVM are absent, making diagnosis difficult. In this report, peculiar MR features in the presence of a connecting vessel between the normal deep venous system of the lower extremity and the tumour provide a clue for the early diagnosis of primary intraosseous AVM.     

Dural metastasis of nasopharyngeal carcinoma: rare,but worth considering

Metastasis of nasopharyngeal carcinoma (NPC) to the dura, an extremely rare condition, can be symptomatically silent and mistaken for a benign entity radiographically. Missed diagnosis can lead to serious consequences or prove immediately fatal. We report a woman with dural metastasis of NPC that mimicked a meningioma on radiography. Craniectomy with tumour resection was performed due to rapid progression from the onset of symptoms to disability. The patient was still alive two years after surgery. This case emphasises the need to keep in mind the possibility of dural metastasis of NPC in patients with abnormal imaging features. This would not only avoid wrong and optimistic diagnosis, but also allow for appropriate treatment in a timely manner. To our knowledge, this is the first report of metastasis of NPC to the dura. We provide detailed information on the neoplastic lesion, which masqueraded as a benign entity and caused potentially fatal consequences.     

Granular cell tumour: malignant or benign?

INTRODUCTION

Granular cell tumours (GrCTs) are uncommon soft tissue tumours that are usually benign (approximately 0.5%–2.0% have been reported as malignant). They are very rarely found at the extremities. Differentiating a malignant GrCT from a benign one is important as the former is aggressive and has a poor prognosis, whereas the latter, after surgical resection, has excellent outcomes. A malignant lesion can be suspected on clinical presentation and confirmed via histopathological examination using the Fanburg-Smith criteria.

METHODS

This was a retrospective review of all cases of GrCT of the extremities that presented to the Orthopaedic Oncology Unit of University Malaya Medical Centre, Malaysia, from September 2006 to March 2013.

RESULTS

There were a total of five cases, all of which involved female patients aged 13–40 (mean age 24) years. Three cases involved the upper limbs and two involved the lower limbs. Using the Fanburg-Smith criteria, three cases were classified as benign, one as atypical and one as malignant. Wide local excision was performed in all five cases and the outcomes were excellent except for the patient with a malignant tumour. That patient presented with lung metastasis about three months after surgery.

CONCLUSION

Malignant and benign GrCTs can be differentiated on clinical presentation and by using the Fanburg-Smith criteria. We believe that wide local excision is the best treatment for both benign and malignant tumours. The role of adjuvant chemotherapy and radiotherapy in malignant GrCTs should be studied. All patients with GrCTs should receive follow-up to check for recurrence and metastasis.     

ECG series. Importance of QT interval in clinical practice

Long QT interval is an important finding that is often missed by electrocardiogram interpreters. Long QT syndrome (inherited and acquired) is a potentially lethal cardiac channelopathy that is frequently mistaken for epilepsy. We present a case of long QT syndrome with multiple cardiac arrests presenting as syncope and seizures. The long QTc interval was aggravated by hypomagnesaemia and drugs, including clarithromycin and levofloxacin. Multiple drugs can cause prolongation of the QT interval, and all physicians should bear this in mind when prescribing these drugs.     

Production of a fragment of glycoprotein G of herpes simplex virus type 2 and evaluation of its diagnostic potential

INTRODUCTION

Herpes simplex virus type 2 (HSV-2) is the most common cause of genital herpes. Glycoprotein G (gG) is a prototype antigen for type-specific serodiagnosis distinguishing between HSV type 1 (HSV-1) and HSV-2 infections. As immunological diagnosis kits for accurate differentiation between HSV-1 and HSV-2 antibodies can be expensive, there is a need to develop a convenient, sensitive, specific and cost-effective serodiagnostic kit.

METHODS

We successfully expressed a fragment of gG comprising residues 321–580 of HSV-2 with histidine tag (gG_321–580His) in a Bac-to-Bac baculovirus expression system, which had an antigenicity similar to its native counterpart. An indirect enzyme-linked immunosorbent assay (ELISA) was developed using gG_321–580His as the diagnostic antigen and evaluated by comparison with a commercial HerpeSelect 2 ELISA immunoglobulin G kit as reference.

RESULTS

In testing 318 field serum samples, the diagnostic relative sensitivity and specificity of the developed gG_321–580His-ELISA test in qualitative comparison with the commercial kit were 93.81% and 96.74%, respectively, and the accuracy was 94.65%.

CONCLUSION

The study indicates that gG_321–580His has a high diagnostic potential for HSV-2 virus serodiagnosis in humans.     

Clinics in diagnostic imaging (159). Jejunal intussusception due to Peutz-Jeghers syndrome.

A 21-year-old woman presented with acute onset of upper abdominal pain. A diagnosis of Peutz-Jeghers syndrome (PJS) was made based on the clinical picture of perioral pigmentation with imaging findings of transient jejunojejunal intussusceptions and small bowel polyps, and confirmed by characteristic histopathological appearances of Peutz-Jeghers polyps. PJS is a rare hereditary condition characterised by unique hamartomatous polyps, perioral mucocutaneous pigmentations, and increased susceptibility to gastrointestinal and extraintestinal neoplasms. Patients usually present with recurrent abdominal pain due to intussusception caused by polyps. Other modes of presentations include rectal bleeding and melaena. We describe the imaging findings of PJS and provide a brief review of bowel polyposis syndromes. The latter are relatively rare disorders characterised by multiple polyps in the large or small intestine, with associated risk of malignancies and other extraintestinal manifestations. Awareness of the manifestations and early diagnosis of these syndromes is crucial to prevent further complications.     

Melorheostosis of the hand affecting the c6 sclerotome and presenting with carpal tunnel syndrome

Melorheostosis is a rare, progressive bone disease accompanied by hyperostosis and soft tissue fibrosis. While affected adults present with contracture and pain, children present with limb length discrepancy and deformity. We report the case of a 20-year-old woman with melorheostosis since childhood who presented with right hand deformity and numbness. Radiographs showed not only a combination of dense sclerosis and opacities, but also the classic ‘flowing candle wax’ appearance. Radiography can be used to identify melorheostosis, thus preventing unnecessary bone biopsies. Carpal tunnel release revealed the presence of a thickened flexor retinaculum and a degenerated median nerve distal to the retinaculum, but did not show hyperostosis. This case highlights the role of nerve decompression in melorheostosis and the importance of early identification of the disease to prevent unnecessary bone biopsies.     

Syndecan-1 is a potential biomarker for triple-positive breast carcinomas in Asian women with correlation to survival

INTRODUCTION

While overexpression of syndecan-1 has been associated with aggressive breast cancer in the Caucasian population, the expression pattern of syndecan-1 in Asian women remains unclear. Triple-positive breast carcinoma, in particular, is a unique subtype that has not been extensively studied. We aimed to evaluate the role of syndecan-1 as a potential biomarker and prognostic factor for triple-positive breast carcinoma in Asian women.

METHODS

Using immunohistochemistry, staining scores of 61 triple-positive breast carcinoma specimens were correlated with patients’ clinicopathological variables such as age, ethnicity, tumour size, histological grade, lymph node status, lymphovascular invasion, associated ductal carcinoma in situ grade, recurrence and overall survival.

RESULTS

Syndecan-1 had intense staining scores in triple-positive invasive ductal breast carcinomas when compared to normal breast tissue. On multivariate analysis, syndecan-1 epithelial total percentage and immunoreactivity score showed statistical correlation with survival (p = 0.02).

CONCLUSION

The intense staining scores of syndecan-1 and their correlation with overall survival in patients with triple-positive breast carcinoma suggest that syndecan-1 may have a role as a biological and prognostic marker in patients with this specific subtype of breast cancer.     

Extraskeletal osteosarcoma arising in the buttock

Extraskeletal osteosarcoma is a rare sarcoma that accounts for about 1% of malignant soft tissue tumours. We report an very unusual case of a small-size extraskeletal osteosarcoma arising in the superficial subcutaneous region of the buttock. The patient was a 55 year-old female. She incidentally noticed a small nodule in the right trochanteric region. It was removed under local anesthesia at a private clinic. An additional wide excision was performed at our hospital. There was an 8 mm? 10 mm mass in the subcutaneous tissue. No invasion of the underlying fascia was observed. Microscopic examination of the removed tumour showed bizarre-looking spindle and giant cells with lace-like osteoid. The tumour was diagnosed as extraskeletal osteosarcoma. Chemotherapy with Rosen T-20 was administered to the patient. The patient has survived without recurrence or metastasis for 4 years since the primary surgery. Its superficial location, very small size, wide excision, and chemotherapy were thought to contribute to her long survival.     

Magnetic resonance imaging of diabetic foot complications

This pictorial review aims to illustrate the various manifestations of the diabetic foot on magnetic resonance (MR) imaging. The utility of MR imaging and its imaging features in the diagnosis of pedal osteomyelitis are illustrated. There is often difficulty encountered in distinguishing osteomyelitis from neuroarthropathy, both clinically and on imaging. By providing an accurate diagnosis based on imaging, the radiologist plays a significant role in the management of patients with complications of diabetic foot.     

Clinics in diagnostic imaging (151). Acromioclavicular joint geyser sign with chronic full-thickness supraspinatus tendon (SST) tear.

An 82-year-old man presented with neck pain, right upper limb radiculopathy and right shoulder pain. Physical examination revealed a soft lump over the right shoulder joint, as well as reduced range of shoulder movements. On magnetic resonance imaging, the soft lump was shown to be a cystic mass over the acromioclavicular joint and was related to a full-thickness supraspinatus tendon tear. This is the classic geyser sign. The pathophysiology and clinical features of the geyser sign, and its imaging features with various imaging modalities, are discussed.     

Lymphocytic colitis complicated by a mass in the terminal ileum

Lymphocytic colitis is a chronic inflammatory disease affecting the bowel. The clinical course of lymphocytic colitis is believed to be benign with watery diarrhoea. We report herein what is, to the best of our knowledge, the first case of lymphocytic colitis complicated by a terminal ileal mass. A 23-year-old man presented with diarrhoea. Blind biopsies of samples taken from the terminal ileum, caecum and ascending colon showed features of lymphocytic colitis. He declined treatment with budesonide or 5-aminosalicylates. He presented 14 months later with pain over the right lumbar region and nausea. Computed tomographic enteroclysis showed a focal soft tissue enhancing mass at the terminal ileum. Excision of the soft tissue mass revealed that it was reactive nodular lymphoid hyperplasia with fibrous granulation tissue. In conclusion, an untreated lymphocytic colitis may result in the formation of an inflammatory mass lesion.     

Intramedullary compression device for proximal ulna fracture

Proximal ulna fractures account for 20% of all proximal forearm fractures. Many treatment options are available for such fractures, such as cast immobilisation, plate and screw fixation, tension band wiring and intramedullary screw fixation, depending on the fracture pattern. Due to the subcutaneous nature of the proximal forearm, it is vulnerable to open injuries over the dorsal aspect of the proximal ulna. This may in turn prove challenging, as it is critical to obtain adequate soft tissue coverage to reduce the risk of implant exposure and bony infections. We herein describe a patient with a Gustillo III-B open fracture of the proximal ulna, treated with minimally invasive intramedullary screw fixation using a 6.0-mm cannulated headless titanium compression screw (FusiFIX™, Péronnas, France).     

Subcutaneous abscess due to Pyrenochaeta romeroi in a renal transplant recipient

An infective aetiology, including fungal infection, should be considered in the differential diagnosis of immunocompromised patients presenting with skin lesions. Dematiaceous fungi are recognised as pathogens in organ transplant recipients. Herein, we describe a rare case of a chronic necrotising granulomatous skin lesion caused by Pyrenochaeta romeroi in a renal transplant recipient, and review the existing literature on the topic. To the best of our knowledge, this is the first report of such a case in Singapore. Recognition of infections caused by dematiaceous fungi is important because some strains are difficult to identify and require special molecular diagnostic techniques. Treatment involves surgical excision and long-term antifungal therapy. Data on the optimal antifungal regimen in such a diagnosis is limited.     

Detection of Strongyloides stercoralis infection among cancer patients in a major hospital in Kelantan,Malaysia

INTRODUCTION

Strongyloidiasis is one of the most commonly neglected but clinically important parasitic infections worldwide, especially among immunocompromised patients. Evidence of infection among immunocompromised patients in Malaysia is, however, lacking. In this study, microscopy, real-time polymerase chain reaction (PCR) and enzyme-linked immunosorbent assays (ELISAs) were used to detect Strongyloides stercoralis (S. stercoralis) infection among cancer patients in a Malaysian hospital.

METHODS

A total of 192 stool and serum samples were collected from cancer patients who were receiving chemotherapy with or without steroid treatment at a hospital in northeastern Malaysia. Stool samples were examined for S. stercoralis using parasitological methods and real-time PCR. Serology by ELISA was performed to detect parasite-specific immunoglobulin G (IgG), IgG4 and immunoglobulin E (IgE) antibodies. For comparison, IgG4- and IgG-ELISAs were also performed on the sera of 150 healthy individuals from the same area.

RESULTS

Of the 192 samples examined, 1 (0.5%) sample was positive for S. stercoralis by microscopy, 3 (1.6%) by real-time PCR, 8 (4.2%) by IgG-ELISA, 6 (3.1%) by IgG4-ELISA, and none was positive by IgE-ELISA. In comparison, healthy blood donors had significantly lower prevalence of parasite-specific IgG (2.67%, p < 0.05) and IgG4 (2.67%, p < 0.05) responses.

CONCLUSION

This study showed that laboratory testing may be considered as a diagnostic investigation for S. stercoralis among immunocompromised cancer patients.     

Laparoscopic grasper for intramedullary biopsy: a technique to improve tissue sampling

Reaming samples are usually sent for histological testing to establish or confirm the primary source of malignancy while intramedullary nailing is performed for impending or established pathological fracture of the long bones. However, this conventional technique of sampling does not always yield accurate results. Herein, we introduce a technique for intramedullary biopsy using a laparoscopic grasper under image intensifier guidance. This novel technique was applied in three cases, and the tissue samples obtained successfully established or confirmed the primary source of malignancy. In comparison, reaming samples obtained via the conventional method showed negative findings in one case and tumour cells that were of poor sample quality in another case. We opine that this new technique is an effective and reproducible method that could improve tissue sampling and achieve a more reliable histological diagnosis.