A term newborn infant developed hypovolaemic shock shortly after birth. She was pale with gross hepatomegaly. She required multiple boluses of intravenous fluids, blood products as well as inotropic support. Blood investigations showed persistent thrombocytopenia, anaemia and disseminated intravascular coagulopathy (DIC). She also developed heart failure. She finally succumbed on the eleventh day of life. Autopsy revealed haemangiomatosis involving the liver, lungs, gastrointestinal tract, kidneys and adrenals.
Pseudotumours are rare, occurring in 1-2% of severe haemophiliacs. Osseous locations are far less frequent than soft tissue location. We report a case of a 43-year-old man with haemophilia A, who presented with a gradually enlarging left thigh mass for 8 months. There were no constitutional symptoms. Plain radiograph showed an expansile lytic lesion with 'soap-bubble' appearance arising from the left femur diaphysis. On MRI, it appeared as a non-enhancing, multilobulated lesion expanding the medullary and subperiosteal spaces. The mass exhibited concentric ring sign with heterogeneous intermediate signal intensity in the core lesion, reflective of chronic haematoma with blood degradation products of different stages. A diagnosis of haemophilic pseudotumour was made. Hypercalcaemia, however, raised a diagnostic dilemma as bone malignancy needed to be considered. An open excisional biopsy and subsequent amputation confirmed the diagnosis of osseous haemophilic pseudotumour. Nuclear medicine study later revealed a concurrent parathyroid adenoma.
Hematopoietic stem cell transplantation (HCT) utilizing non-myeloablative (NMA) and reduced-intensity conditioning (RIC) regimens (collectively referred to as reduced-toxicity HCT, RT-HCT) has become a viable therapeutic option for patients with hematological malignancies who are ineligible for standard myeloablative conditioning transplantation (MA-HCT). RT-HCT has been shown to induce stable engraftment with low toxicity, and to produce similar overall and progression-free survival (PFS) when compared to MA-HCT in acute myeloid leukemia and myelodysplastic syndrome. The best results for RT-HCT have been reported for patients with disease that is in remission, indolent and chemosensitive, and with a strong graft-versus-malignancy effect. Chronic graft-versus-host disease seems to correlate with a lower relapse rate and better PFS. RT-HCT is inferior when performed in poor risk or advanced disease, due to high relapse rates. A search for novel strategies that includes the most appropriate conditioning regimens and post-transplant immunomodulation protocols with more intensive anti-malignancy activity but limited toxicity is in progress. This review provides an update on the results of clinical studies of RT-HCT, and discusses possible indications and investigative strategies for improving the clinical outcomes of RT-HCT for the major hematological malignancies.
Uterine fibroid or leiomyoma is the commonest benign uterine tumour. Its occurrence in the postmenopausal age group is rare and if enlargement of the fibroid noted during this time, the diagnosis of leiomyosarcoma is provisional until proven otherwise. A case of a postmenopausal woman with a huge uterine fibroid associated with polycythaemia is presented whereby the mass was noted to increase in size within 2 years. The patient was otherwise well except for the growing uterine mass. She had venesection done to treat her polycythaemia and later underwent surgery for total abdominal hysterectomy and bilateral salpingoophoorectomy. The histopathology report confirmed leiomyoma of uterus with no evidence of sarcomatous changes. It was suggested that large uterine myoma may cause secondary polycythaemia by three postulated mechanisms, i.e. presence of hypoxia suggesting shunting within the tumour, second, the uterine fibroid was compressing the ureters resulting in inappropriate excessive production of erythropoietin by the kidneys, and third, the tumour itself may have been producing the erythropoietin.
A 17-year-old, sexually active, single, nulliparous young woman presented to us with one week history suggestive of nephrotic syndrome. She was found to have a benign hydatidiform mole confirmed by histopathological examination after suction and curettage. Renal biopsy revealed focal segmental glomerulosclerosis. The renal pathology was most probably due to molar pregnancy due to the close temporal relationship. To our knowledge, this is the first case of focal segmental glomerulosclerosis associated with a gestation trophoblastic disease described in the literature.
Endometriosis and its complications are not uncommon in Malaysian women. Two cases of ureteric obstruction due to endometriosis are reported and the literature on ureteric obstruction and endometriosis reviewed. It is emphasized that the possibility of ureteric obstruction must be suspected when there is extensive endometriotic disease or if the patient presents with loin pain or pain on one side of the abdomen. Ultrasound scan of the kidneys and, if necessary, an intravenous pyelogram will have to be done on the slightest suspicion. Patients with endometriosis will have to be followed up until the menopause or even later if they are on hormone replacement therapy.
Anti-N-methyl-d-aspartate receptor encephalitis is a recently described neurological disorder and an increasingly recognized cause of psychosis, movement disorders and autonomic dysfunction. We report 20-year-old Chinese female who presented with generalized tonic-clonic seizures, recent memory loss, visual hallucinations and abnormal behavior. Anti-N-methyl-d-aspartate receptor encephalitis was diagnosed and a computed tomography scan of abdomen reviewed a left adnexal tumor. We describe the first such case report of a patient with anti-N-methyl-d-aspartate receptor encephalitis who was given a bilateral transversus abdominis plane block as the sole anesthetic for removal of ovarian tumor. We also discuss the anesthetic issues associated with anti-N-methyl-d-aspartate receptor encephalitis. As discovery of tumor and its removal is the focus of initial treatment in this group of patients, anesthetists will encounter more such cases in the near future.
We report a case of a 45 year-old man who presented initially with a non-functioning pituitary macroadenoma. A routine chest radiography done preoperatively revealed a right lung nodule which was confirmed by computed tomography (CT) of the thorax. Transfrontal hypophysectomy was performed while a conservative approach was taken for the lung nodule. Four years later, he presented acutely with adrenocorticotrophic hormone (ACTH) dependent Cushing's syndrome which resolved following a right lobectomy. Histological examination revealed an atypical carcinoid. To our knowledge, this is the first reported case of an ectopic ACTH secreting pulmonary carcinoid found in association with a non-functioning pituitary macroadenoma.
A patient who presented with acute intestinal obstruction had a right hemicolectomy for a caecal tumour. The histopathology report confirmed metastatic carcinoma in the caecum from the cervical carcinoma. Caecum is a very rare site of metastasis from cervical carcinoma. From our literature review, there have been no such cases reported.
Osteosarcoma is the most common primary malignant tumour of bone and it usually metastasises to the lung. In the nasal cavity metastatic disease is extremely rare. We describe a case of osteosarcoma presenting with recurrent epistaxis, and proptosis due to secondaries in the nasal cavity. To our knowledge such a case has not been reported previously in the available English literature.
Matched MeSH terms: Nose Neoplasms/complications; Skull Neoplasms/complications
A case of an adenoid cystic carcinoma of the minor salivary glands of then nasal cavity is reported. The tumour had spread locally and by perineural spread to the internal auditory mentus, causing facial nerve palsy.
Matched MeSH terms: Nose Neoplasms/complications*; Salivary Gland Neoplasms/complications*
A 39-year-old man was diagnosed with von Hippel-Lindau syndrome, which was associated with retinal haemangioblastoma, cervical cord haemangioblastoma and bilateral renal cell carcinoma. He subsequently underwent an arterial embolisation and cervical laminectomy, following a spinal angiogram of the cervical lesion. He also had a right radical nephrectomy, with no perioperative complications. However, on admission for the left radical nephrectomy, he was noted to have preoperative hypertension. Further investigation revealed an enlarged left adrenal gland on abdominal computed tomography scan and raised urinary catecholamines. We discuss the risk of renal cell carcinoma and phaeochromocytoma arising concomitantly in von Hippel-Lindau syndrome, and how best to investigate and manage them.
A 72-year-old man was referred from the surgery department for rehabilitation following surgical resection of Basaloid carcinoma. The first surgical intervention involved the anterior palatal region and was restored with a simple obturator. Two years later further surgery was undertaken to excise a recurrent tumor in the nose and part of the cheek. This resulted in an exposed nasal cavity and maxillary sinus. In addition, there was a small oral aperture composed of thin tissue that stretched to its maximum due to scar formation. The defect was restored with a full thickness skin flap but it subsequently broke down leaving the midface exposed with limited mouth opening due to tissue contraction and scar formation after the flap operation. The defect was rehabilitated with Co-Cr obturator intraorally and a silicone nose retained to the naso-palatal extension of the obturator by a magnet extraorally. This resulted in practically good retention, placement, and adaptation of the two parts of the prosthesis.
Matched MeSH terms: Maxillary Sinus Neoplasms/complications; Nose Neoplasms/complications; Palatal Neoplasms/complications
Breast carcinoma is the most common primary tumor producing intraocular metastasis. Metastases to the iris and ciliary body are relatively rare. The authors report a case of a 61-year-old lady, operated for carcinoma of the left breast 3 years back, who presented with symptoms and signs of acute narrow-angle glaucoma in the right eye. A diffuse whitish plaque-like mass in the upper nasal quadrant of the iris with an episcleral nodule on the limbus in the corresponding area and all the signs of acute narrow-angle glaucoma were present in the right eye. Intraocular pressure was controlled medically. Fine-needle aspiration cytology from the episcleral nodule showed malignant cells. Histopathology of the excised nodule showed metastatic poorly differentiated carcinoma, and the cellular pattern was similar to the carcinoma of the breast. There was no other metastasis anywhere in the body. Fine-needle aspiration cytology from an external lesion of the eye is a less invasive and easier procedure than paracentesis to diagnose the metastatic nature of the lesions. The rare features in our case are the clinical presentation as acute glaucoma and the ocular structures being the first and only site of metastasis.
Matched MeSH terms: Breast Neoplasms/complications; Eye Neoplasms/complications; Uveal Neoplasms/complications
BACKGROUND:
Cognitive impairment, colloquially termed "chemobrain", occurs in 10-40% of all cancer patients, and is an emerging target of cancer survivorship research.
AIM:
This study reviews published qualitative studies to explore cognitive impairments or chemobrain among breast cancer survivors, with particular attention given to the impact on quality of life.
METHOD:
Using keywords, we searched ten electronic databases (CINAHL, EMBASE, Proquest, OVID SP, MEDLINE, Oxford Journal, Science Direct, PubMED).
FINDINGS:
Of 457 papers, seven relevant papers were included. Data was extracted and concepts were analysed using a meta ethnography approach. Four second order intepretations were identified, on the basis of which, four third order intrepretations were constructed. Linked together in a line of argument, was a consistent account on their struggles to self-manage the chemobrain impairments that impact their daily lives. Five concepts emerged from the analysis of the primary findings: i) real experiences of cognitive changes, ii) calls for help, iii) impact of cognitive impairments, iv) coping and v) survivorship and meaning. Further synthesis resulted in four new order intepretations: i) The chemobrain struggle, ii) The substantial impact of chemobrain on life domains, iii) The struggle to readjust and to self manage, and iv) 'thankful yet fearful' representation.
DISCUSSION:
Awareness of cognitive changes were context-dependent on healthcare settings and cultural contexts as strong determinants. Subjects verified the existence of chemobrain but healthcare providers mis-recognised, under-recognised, and sometimes negated it perhaps due to its unknown aetiology. Asian breast cancer survivors appear less vocal than their western counterparts.
CONCLUSION:
The current literature on the lived experiences of how women experienced chemobrain provides a consistent report that chemobrain is real, persistent and with detrimental impacts on quality of life - manifested as a constant struggles. A greater awareness of the effects of chemobrain with improved functional assessment and interventions is warranted.
Matched MeSH terms: Breast Neoplasms/complications*
Involvement of a multidisciplinary team in cancer care may have added benefits over the existing system of patient management. A paradigm shift in the current patient management would allow more focus on nutritional support, in addition to clinical care. Malnutrition, a common problem in cancer patients, needs special attention from the early days of cancer care to improve quality of life and treatment outcomes. Patient management teams with trained oncology dietitians may provide quality personalized nutritional care to cancer patients.