Kaposi Sarcoma of the Face Not Associated with Acquired Immunodeficiency Syndrome: A Case Report and Review of the Literature

Kaposi sarcoma (KS) is an angioproliferative vascular neoplasm characterized by the proliferation of endothelial cells that is typically seen in patients with acquired immunodeficiency syndrome (AIDS). There are four major subtypes of KS: classic, African endemic, iatrogenic, and AIDS-associated KS. In rare circumstances, a patient might present with KS that does not fit into any of the four major subtypes and has no identifiable predisposing factors. This case report and review of the literature is presented to serve as a reminder to practitioners to suspect the unexpected when considering cystic or vascular-type lesions on the head and neck.     

Liver transplantation for children: Red Cross Children's Hospital experience

BACKGROUND: The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. METHODS: Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed < 10 kg. RESULTS: Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.     

Kaposi Sarcoma of the Medial Foot in an MSM,HIV-Negative Man: A Fifth Clinical Variant

Kaposi sarcoma (KS) is a low-grade vascular neoplasm with four well-documented clinical types: classic, African (endemic), acquired immunodeficiency syndrome (AIDS)-associated (epidemic), and iatrogenic, as well as a fifth recently described variant, men having sex with men (MSM) without human immunodeficiency virus (HIV) infection. Often distinguishable on clinical exam, KS lesions are typically found on the skin and mucosa of immunocompromised individuals, though numerous published reports have demonstrated the ability of KS to present in immunocompetent individuals. Here, we present the case of a 66-year-old, HIV-negative MSM with violaceous, non-blanching macules and patches of the medial foot referred to dermatology by his primary care physician. Histopathologic analysis revealed Kaposi sarcoma. Although this patient engaged in same-sex sexual behaviors, his HIV-negative status and otherwise lack of immunocompromise would deter many clinicians from suspecting a diagnosis of KS. Despite therapy, disease prognosis is often dependent upon stage at diagnosis. Therefore, it is important that every clinician understand the cutaneous findings associated with KS and be aware of the higher rates of KS even in HIV-seronegative MSM to facilitate prompt diagnosis and immediate treatment of this high-risk population.     

Excellent clinical outcomes and retention in care for adults with HIV-associated Kaposi sarcoma treated with systemic chemotherapy and integrated antiretroviral therapy in rural Malawi

Introduction

HIV-associated Kaposi sarcoma (HIV-KS) is the most common cancer in Malawi. In 2008, the non-governmental organization, Partners In Health, and the Ministry of Health established the Neno Kaposi Sarcoma Clinic (NKSC) to treat HIV-KS in rural Neno district. We aimed to evaluate 12-month clinical outcomes and retention in care for HIV-KS patients in the NKSC, and to describe our implementation model, which featured protocol-guided chemotherapy, integrated antiretroviral therapy (ART) and psychosocial support delivered by community health workers.

Methods

We conducted a retrospective cohort study using routine clinical data from 114 adult HIV-KS patients who received ART and ≥1 chemotherapy cycle in the NKSC between March 2008 and February 2012.

Results

At enrolment 97% of patients (n/N=103/106) had advanced HIV-KS (stage T1). Most patients were male (n/N=85/114, 75%) with median age 36 years (interquartile range, IQR: 29–42). Patients started ART a median of 77 days prior to chemotherapy (IQR: 36–252), with 97% (n/N=105/108) receiving nevirapine/lamivudine/stavudine. Following standardized protocols, we treated 20 patients (18%) with first-line paclitaxel and 94 patients (82%) with bleomycin plus vincristine (BV). Of the 94 BV patients, 24 (26%) failed to respond to BV requiring change to second-line paclitaxel. A Division of AIDS grade 3/4 adverse event occurred in 29% of patients (n/N=30/102). Neutropenia was the most common grade 3/4 event (n/N=17/102, 17%). Twelve months after chemotherapy initiation, 83% of patients (95% CI: 74–89%) were alive, including 88 (77%) retained in care. Overall survival (OS) at 12 months did not differ by initial chemotherapy regimen (p=0.6). Among patients with T1 disease, low body mass index (BMI) (adjusted hazard ratio, aHR=4.10, 95% CI: 1.06–15.89) and 1 g/dL decrease in baseline haemoglobin (aHR=1.52, 95% CI: 1.03–2.25) were associated with increased death or loss to follow-up at 12 months.

Conclusions

The NKSC model resulted in infrequent adverse events, low loss to follow-up and excellent OS. Our results suggest it is safe, effective and feasible to provide standard-of-care chemotherapy regimens from the developed world, integrated with ART, to treat HIV-KS in rural Malawi. Baseline BMI and haemoglobin may represent important patient characteristics associated with HIV-KS survival in rural sub-Saharan Africa.     

HIV-related primary Kaposi's sarcoma of the lower eyelids mimicking a vascular malformation in an otherwise asymptomatic patient

A patient presenting with reddish-blue nodules on both the lower eyelids and no other lesion is reported. The nodules were compressible and promptly resumed their original size and shapes upon removing the pressure. At first sight, these lesions were clinically suggestive of a vascular malformation. Carotid angiograms supported this diagnosis. However, the analysis of the serum samples of the patient revealed an underlying HIV infection. Therefore, a diagnosis of Kaposi's sarcoma (KS) was considered as a possibility. Incisional biopsy histology and immunohistochemistry confirmed the diagnosis of KS. In this case, the clinical presentation, the angiography, and the absence of any other symptoms suggestive of an immunodeficient status were clearly misleading and the correct clinical diagnosis was only made possible by the laboratory studies.     

Profile of children with head injuries treated at the trauma unit of Red Cross War Memorial Children's Hospital, 1991 - 2001.

OBJECTIVE: To describe the profile of childhood head injury patients treated in a trauma unit. DESIGN: A retrospective record-based study. SETTING: The trauma unit of the Red Cross War Memorial Children's Hospital. SUBJECTS: Children (under 13 years of age) presenting with head injuries between January 1991 and December 2001. RESULTS: Of the almost 94 000 records, more than one-third were children presenting with head injuries. Fifty-nine per cent were boys, with more than half the sample under 5 years of age. The majority of children presented with superficial lacerations and abrasions, mostly affecting the scalp and skull. Injuries were mainly caused by falls from a variety of heights, and traffic-related injuries. Almost two-thirds of traffic-related injuries involved children as pedestrians being struck by a motor vehicle. More than 60% of injuries occurred in or around the child's own home. CONCLUSIONS: Head injuries in children are a significant cause of morbidity. Prevention, especially in the home and on the streets, needs urgent attention.     

Antiretroviral therapy and Kaposi’s sarcoma trends and outcomes among adults with HIV in Latin America

IntroductionKaposi’s sarcoma (KS) remains the most frequent malignancy in persons living with HIV (PWH) in Latin America. We examined KS trends and outcomes from Latin American clinical sites in the era of increased access to antiretroviral therapy (ART).MethodsCohorts in Brazil, Peru, Mexico, Honduras, Argentina and Chile contributed clinical data of PWH ≥16 years old from 2000 to 2017, excluding patients with KS diagnosed before clinic enrolment. We compared KS incidence over time using multivariable incidence rate ratios. Predictors of KS before/at or after ART initiation and of mortality after KS were examined using Cox regression.ResultsOf 25 981 PWH, 481 had incident KS, including 200 ART‐naïve and 281 ART‐treated patients. From 2000 to 2017, the incidence of KS decreased from 55.1 to 3.0 per 1000 person‐years. In models adjusting for CD4 and other factors, the relative risk for KS decreased from 2000 to 2008. Since 2010, the adjusted risk of KS increased in the periods before and ≤90 days after ART initiation but decreased >90 days after ART. In addition to low CD4 and male‐to‐male sex, KS risk after ART was associated with age and history of other AIDS‐defining illnesses. Mortality after KS (approximately 25% after five years) was not associated with either year of KS diagnosis nor timing of diagnosis relative to ART initiation.ConclusionsKS incidence in Latin America has remained stable in recent years and risk is highest before and shortly after ART initiation. Early diagnosis of HIV and ART initiation remain critical priorities in the region.     

Cystic undifferentiated sarcoma of liver in children: a pitfall diagnosis in endemic hydatidosis areas

Undifferentiated (embryonal) sarcoma (UES) of the liver is a very rare pediatric neoplasm with an aggressive behavior. Multimodal treatment including complete surgical resection and chemotherapy has improved survival.We report a case of UES occurring in a 15-year-old female. Radiographic findings showed a multicystic mass appearance. The diagnosis of hydatid cyst was initially presumed. A complete tumor resection after 3 cycles of chemotherapy was performed. Histologically, only 20% of tumor necrosis was noted. Adjuvant chemotherapy was instituted. She remained well at 11 months of follow-up.Cystic appearance is a usual finding in UES. Differential diagnosis with hydatid cyst may be problematic in endemic hydatidosis areas.     

The incidence of soft tissue sarcoma in Dakshina Kannada: study in a District Government Hospital

Background  

We present a retrospective study depicting the incidence and outcome of soft tissue sarcomas (STSs) in patients admitted in a District Government Hospital situated in coastal belt of Southern India for a period of four and a half years. The hospital is a district referral centre catering to rural and urban poor population of 1,900,000 people.     

Undifferentiated embryonal sarcoma in children: beware of the solitary liver cyst

Undifferentiated embryonal sarcoma (UES) of liver is a rare form of liver tumor in children. It may have an atypical presentation leading to delay in diagnosis. The authors report on 3 consecutive children with this condition, each of them presenting in older children who presented with this condition and an initial diagnostic dilemma. One of them presented initially with a solitary cyst, the second with severe abdominal pain, and the third with intermittent fever. Preoperative chemotherapy was successful in shrinking the tumors in 2 children to allow anatomic liver resection with tumor-free margin. Both remain free of recurrence 2 years after completing treatment.     

Clear cell sarcoma arising from the chest wall: a case report

Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The clinical course is rather slow, with repeated local recurrences followed by late metastases and eventual death. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. We report a case of clear cell sarcoma arising from the chest wall. The patient, a 20-year-old woman, had noticed a chest wall mass and pain for 2 years. Biopsy of the mass showed abundant nests of round cells with clear cytoplasm. On immunohistochemical examination, tumor cells were strongly immunoreactive for S-100 and HMB-45. A diagnosis of clear cell sarcoma was confirmed. There was no other lesion found in the patient through routine imaging studies. She was treated with two courses of chemotherapy using ifosfamide, carboplatin, and etoposide. Subsequently, the tumor, including adjacent tissue, the chest wall, and sternum, was resected with a wide margin; and the defect of the chest wall was covered with Marlex mesh fabric, regin, and a musculocutaneous flap. She has shown no symptoms or signs of recurrence during 2 years of follow-up.     

Small-bowel myeloid sarcoma: Report of a case with atypical presentation

INTRODUCTION

Small-bowel myeloid sarcoma is rare. Acute bowel obstruction is its usual clinical presentation.

PRESENTATION OF CASE

We report a case of small-bowel myeloid sarcoma that occurred in a 64-year-old woman who presented chronic secretory diarrhoea, hypokalaemia, and weight loss. Immature white blood cells in a peripheral smear and small-bowel capsule endoscopic features were the main diagnostic clues. The patient experienced capsule retention and developed acute bowel obstruction. Urgent laparotomy showed a stricturing ileal mass and pathology of the resected bowel specimen unveiled a CD34+, CD117+, and myeloperoxidase-positive myeloid sarcoma. The diarrhoea promptly resolved after surgery, and the patient is now undergoing chemotherapy.

DISCUSSION

Secretory diarrhoea can be the first manifestation of small-bowel myeloid sarcoma. Capsule endoscopy may provide a diagnostic clue, but it can trigger an acute bowel obstruction. Differential diagnosis of the pathologic specimen may be difficult and a high suspicion index of is mandatory to perform immunophenotyping to determine the correct management.

CONCLUSION

Chronic diarrhoea with alarm features can be the first manifestation of small-bowel myeloid sarcoma.Abbreviations: AML, acute myeloid leukaemia; MS, myeloid sarcoma; SBCE, small bowel capsule endoscopy