Misdiagnosed: A Sigmoid Perforation Secondary to Visceral Kaposi Sarcoma.

In a case-control study, we studied the effect of a single nucleotide polymorphism in the IL-8 promoter on the risk of the development of AIDS-related Kaposi's sarcoma (KS). KS developed in 46% of individuals with the TT genotype and in 66% of AA/AT genotypes (P=0.038). Patients with TT genotype were rarely affected with visceral KS (7% versus 36%; P=0.06), which suggests that carriers of the TT genotype are protected from (severe) KS development.

The incidence of Kaposi's sarcoma among recipients of solid organs is about 500 times the rate in the general population, suggesting a role for immunosuppression in its development. On the basis of these findings, we investigated the impact of sirolimus on cutaneous and disseminated visceral Kaposi's sarcoma in a renal-transplant recipient. The introduction of sirolimus in this patient allowed complete regression of Kaposi's sarcoma (cutaneous and visceral) with preservation of excellent renal function. Meanwhile, in view of the available observational reports, we think that sirolimus should be included in the standard treatment for Kaposi's sarcoma after transplantation, to permit remission of the sarcoma (both cutaneous and visceral) while preserving the renal function.

Epidemic Kaposi's sarcoma remains the most common cancer in patients with human immunodeficiency virus and is associated with significant morbidity and mortality in AIDS patients. Primary visceral Kaposi's sarcoma (Kaposi's sarcoma without cutaneous lesions) presenting with lower gastrointestinal bleeding (LGIB) has rarely been reported. Though Kaposi's sarcoma can occur anywhere in gastrointestinal tract, gastrointestinal symptoms are often non-specific such as chronic blood loss anaemia, vomiting, diarrhoea, intestinal obstruction. In these patients, severe gastrointestinal bleeding requiring repeated blood transfusions is extremely rare. Clinicians should be aware of gastrointestinal tract Kaposi's sarcoma since visceral Kaposi's sarcoma can present in the absence of cutaneous involvement. Endoscopy with biopsy is useful in the diagnosis for severe LGIB in patients with AIDS. Furthermore, gastrointestinal Kaposi's sarcoma should be considered in the differential diagnosis of GI bleeding. We report a case of primary colonic KS who presented with recurrent GI bleeding which was eventually diagnosed by sigmoidoscopy and confirmed pathologically.

Kaposi's sarcoma is an uncommon neoplasm that occasionally involves the gastrointestinal tract in immunosuppressed individuals. Infection with human herpes virus 8 is known to be necessary for developing all forms of Kaposi's sarcoma. We report a renal transplant recipient who developed visceral Kaposi's sarcoma 18 months after the transplantation. In Oriental countries, the incidence of Kaposi's sarcoma is extremely low, and this is the first case of Kaposi's sarcoma arising from a transplant recipient in Japan. Standard forceps biopsies of the gastric lesions failed to make the correct diagnosis. However, endoscopic resection successfully led to the correct diagnosis of Kaposi's sarcoma and herpes simplex virus 8 infection as well. This is the first report of a patient with visceral Kaposi's sarcoma who underwent endoscopic resection that reliably confirmed histological diagnosis and the viral genome at the same time.

Chylothorax is a rare complication of visceral Kaposi's sarcoma. We report a case with bilateral chylothoraces secondary to relapsed visceral Kaposi's sarcoma who was successfully treated with paclitaxel chemotherapy.

Human herpes virus 8 (HHV8) DNA has recently been detected in sarcoma tissue of patients with Kaposi's sarcoma. HHV8 DNA could also be found in bronchoalveolar lavage (BAL) fluid of patients with tracheobronchial Kaposi's sarcoma. To determine the specificity, sensitivity and predictive values of HHV8 DNA detection in the BAL for the diagnosis of pulmonary Kaposi's sarcoma, 100 consecutive BAL were prospectively analyzed for the presence of HHV8 DNA using a nested PCR assay. In addition, 19 BAL samples of 14 AIDS patients with cutaneous or visceral Kaposi's sarcoma were retrospectively investigated. The prospective group consisted of 79 BAL performed in immunocompromised and of 21 BAL in nonimmunocompromised patients. Four patients of the prospectively analyzed group undergoing six BAL showed tracheobronchial Kaposi's sarcoma at five bronchoscopies. All of the five BAL samples performed in these patients with endoscopically visible Kaposi's sarcoma were positive for HHV8 DNA. Following chemotherapy and antiretroviral treatment tracheobronchial Kaposi's sarcoma was no longer detectable at a subsequent bronchoscopy and HHV8 DNA in BAL became negative in one patient. One BAL sample of a HIV-positive patient with no evidence of Kaposi's sarcoma was HHV8 DNA-positive. The sensitivity, specificity, positive and negative predictive values of HHV8 detection for the diagnosis of tracheobronchial Kaposi's sarcoma were 100%, 98.9%, 83.3%, and 100%, respectively. Twelve of 19 BAL samples of the retrospective group were HHV8 DNA-positive. In this group, 10 patients undergoing a total of 14 BAL suffered from pulmonary Kaposi's sarcoma. HHV8 DNA was documented in 10 of these 14 BAL samples. In three BAL of this group HHV8 DNA was positive, but pulmonary Kaposi's sarcoma was diagnosed at a later stage. In conclusion, the detection of HHV8 DNA in BAL is restricted to patients with Kaposi's sarcoma and is highly sensitive and specific for pulmonary involvement of Kaposi's sarcoma.

Human immunodeficiency virus type 1 in a seronegative patient with visceral Kaposi's sarcoma and hypogammaglobulinemia

We present a case of a patient who is HIV positive and developed both thrombotic thrombocytopenia purpura and visceral Kaposi's sarcoma (KS) with hemorrhage. This case presents a difficult management problem in that the patient's bleeding originated from KS lesions and did not quickly abate with plasmapheresis therapy despite both clinical and laboratory improvement after 2-4 days. Chemotherapy was initiated on day 13 and the patient's condition improved markedly afterward. We believe the addition of chemotherapy to plasmapheresis hastened the improvement of our patient's thrombotic thrombocytopenic purpura (TTP) and KS-related bleeding. Therefore, under similar conditions, we recommend combining plasmapheresis and chemotherapy at the onset of therapy.

Massive hemorrhage from suture line ulceration: documentation by endoscopy

Visceral Kaposi's sarcoma presenting with gingival lesions

Pulmonary Manifestations of Kaposi’s Sarcoma

Trimetrexate glucuronate, a dihydrofolate reductase inhibitor related to methotrexate, was developed by Parke-Davis as an alternative antineoplastic agent for tumors, especially sarcomas, that had developed resistance to methotrexate. This is a report on a patient with AIDS who developed Pneumocystis carinii pneumonia, which was treated with trimethoprim sulfamethoxazole (Bactrim) with poor response, then with pentamidine with poor response, and finally with trimetrexate glucuronate (Neutrexin) and leucovorin rescue, with good response. The patient also suffered from cutaneous and visceral Kaposi's sarcoma (KS), which had been treated with high- dose HCG1 and well recognized chemotherapeutic protocols. Both HCG and chemotherapy resulted in tumor regression. The patient's KS flared, however, when he developed pneumocystis pneumonia. When trimetrexate glucuronate and leucovorin rescue were administered, his tumor burden decreased significantly, suggesting that trimetrexate glucuronate may have some activity against KS. The regression of KS in this anecdotal observation may be secondary to a delayed response from HCG and/or chemotherapy, or secondary to a spontaneous partial regression. Such regression may only be of the decreased edema around the KS lesions and not the neoplastic tissue itself. If other clinicians see this same phenomenon, however, it is possible that trimetrexate glucuronate may have an anti-KS effect. Such future clinical observations would warrant further testing at the basic science level.

The incidence of Kaposi's sarcoma (KS) has increased in solid organ transplantation recipients. This type of KS tends to be aggressive, involving lymph nodes, mucosa and visceral organs in about half of patients, sometimes in the absence of skin lesions. Brain involvement of KS has rarely been reported. A 16-yr-old Turkish boy underwent renal transplantation from his mother. The immunosuppressive regimen included prednisolone, cyclosporin A and azathioprine. Fourteen months later the azathioprine was changed to cyclophosphamide (3 mg/kg/day) because of the development of a nephrotic syndrome. After 12 weeks, the cyclophosphamide was changed to mycophenolate mofetil (MMF) to control the nephrotic syndrome. At this time his serum creatinine level rose to 2.1 mg/dL. Polyclonal or monoclonal antibodies were never given. Multiple intra-abdominal lymphadenopathy was detected on abdominal tomography at the 32nd month after renal transplantation. Kaposi's sarcoma was diagnosed via laparotomy and biopsy. He had a generalized tonic and clonic seizure and contrast enhanced cranial tomography showed two intracranial masses which had an abundant vascular component which caused a mild shift. One of the masses was removed via a burr-hole with the aim of diagnosis and treatment of the shift. A pathologic examination of the intracranial lesion was also reported as Kaposi's sarcoma. Herpes virus-8 DNA was detected by PCR in the intracranial lesion.

Kaposi's sarcoma is a rare neoplasm of characteristic chronicity. The classical form which occurs most often in elderly men of Eastern European origin, comprises both an indolent, cutaneous type marked by spontaneous regression with prolonged survival, and a rarer, disseminated variant is more fulminant. Seven elderly Jewish patients with classical, disseminated, visceral Kaposi's sarcoma were studied; they were neither homosexual nor drug-abusers. All immunologic parameters were normal and serum tests for HIV antibodies, CMV, and EBV were negative. Five of these patients were treated and four responded well, including two complete remissions. The prolonged survival of these patients (82% at 5 years) suggests the existence of an indolent subtype or forme fruste of the usually aggressive form of classical Kaposi's sarcoma.

In the United States, visceral Kaposi's sarcoma most commonly occurs in association with the acquired immune deficiency syndrome (AIDS). The authors report a unique case of primary colonic Kaposi's sarcoma in a young heterosexual HIV-negative man with chronic ulcerative colitis.