|Year : 2018 | Volume
| Issue : 2 | Page : 137-139
Orbital kaposi sarcoma in a female retroviral disease negative nigerian and a review of literature
Rita O Momoh1, Eno A Chude1, Evaristus O Oboh2, Darlington E Obaseki3
1 Department of Ophthalmology, University of Benin Teaching Hospital, Benin City, Nigeria
2 Department of Radiotherapy, University of Benin Teaching Hospital, Benin City, Nigeria
3 Department of Pathology, University of Benin Teaching Hospital, Benin City, Nigeria
|Date of Web Publication||13-Feb-2019|
Dr. Rita O Momoh
Department of Ophthalmology, University of Benin Teaching Hospital, Benin City
Source of Support: None, Conflict of Interest: None
An orbital location of Kaposi sarcoma (KS) is rare, and none has been reported in Nigeria to the best of our knowledge. We report the multidisciplinary management of a case; a 50-year-old human immune-deficiency virus, seronegative female who presented with a painless, progressive swelling of the left eye of two years duration that was histologically confirmed as orbital KS. Occurrence of KS in the orbit is rare; we highlight the need for a very high index of suspicion for this tumor as well as the challenges of managing this unsightly indolent tumor in Nigeria.
Keywords: Female, HIV-seronegative, Kaposi-sarcoma, Nigerian, orbital
|How to cite this article:|
Momoh RO, Chude EA, Oboh EO, Obaseki DE. Orbital kaposi sarcoma in a female retroviral disease negative nigerian and a review of literature. Niger J Ophthalmol 2018;26:137-9
| Introduction|| |
Kaposi sarcoma (KS), first described by the Hungarian dermatologist, Moritz Kaposi in 1872 is a rare, multifocal, vascular lesion of low-grade malignant potential typically presenting in mucocutaneous sites such as the skin of the lower extremities, face, trunk, genitalia, oropharyngeal mucosa, and the eye, which is an unusual location.,, Human herpesvirus 8 (HHV-8) also known as KS-associated herpes virus is believed to be a major causative factor for all clinical variants of KS, but the mechanisms linking infection with this virus to the lesions are rather unknown.
The incidence of Kaposis sarcoma is on the rise following the spread of human immunodeficiency virus/acquired immune-deficiency virus syndrome (HIV/AIDS). Ocular KS has been reported commonly in seropositive individuals as an initial manifestation of HIV infection,,, thus, it is a marker for diagnosis of HIV/AIDS in some patients.
The commonly reported ocular sites include the eyelids, lacrimal gland, and bulbar conjunctiva.,, An orbital location of this tumor occurring in an HIV-seronegative individual is rare and on searching the literature, only a single case was found.
We report the case of a HIV-seronegative woman with orbital KS.
| Case Report|| |
A 50-year-old female farmer from South South Nigeria presented to our clinic with a painless, progressive swelling of the left eye associated with redness of 2 years duration, and total loss of vision 10 months prior to presentation. She admitted to a history of blunt trauma to the eyeball in the farm 10 years before the onset of symptoms. She gave a history of use of traditional medications to treat the progressive ocular mass for a year preceding her presentation at our facility.
On ocular examination, the presenting visual acuity in the right eye was 6/9, anterior and posterior segment findings were essentially normal, and the vertical cup–disc ratio was 0.4 with a healthy looking macula. In the left eye, the presenting visual acuity was no light perception, there was a nonaxial proptosis and slight medial displacement of the left globe which was embedded in a huge orbital mass measuring about 60 × 70 × 40 mm. The mass was firm and tender to touch, with a hyperemic, mucocutaneous (conjunctival) surface with areas of necrosis [Figure 1]. It was not attached to the overlying eye lid skin. The cornea was hazy and keratinized, and there was no view of other structures of the globe. There was no evidence of local spread beyond the left orbit, no peripheral lymph node enlargement, and other systems were essentially normal.
|Figure 1 Photograph showing left ocular mass with fungation and devitalized globe within|
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A cranial computed tomography scan showed an extensive left orbital mass without calcifications or areas of lucency, it was mildly enhancing and isodense, causing anteromedial displacement of the globe with associated expansion of the orbit without gross intracranial extension [Figure 2]. An initial diagnosis of suspected left orbital lymphoma to rule out pleomorphic adenoma of the lacrimal gland was made. All initial hematological tests done including full blood count and peripheral blood film showed no abnormality; however, there was some depression of all cell lines after she had a second course of chemotherapy. The retroviral screening test (Enzyme Linked ImmunoSorbent Assay) was negative for HIV 1 and 2 antibodies on repeated testing and fasting blood sugar was 82 mg/dl.
|Figure 2 Cranial computerized tomogram showing left orbital with no intracranial extension|
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The patient had a wedge incisional biopsy of the orbital mass and marked intraoperative hemorrhage with an estimated blood loss of 500 ml. The tumor was found to be highly vascular and friable, and the nontraumatized tumor surface continued to bleed spontaneously and intermittently weeks after the procedure. However, hematocrit levels remained within normal limits, and she remained constitutionally stable postoperatively.
Histopathological examination of the tissue biopsy showed features in keeping with KS [Figure 3]. External beam radiotherapy (EBR) to the left eye, 40 Gy in 20 fractions over 4 weeks using a direct field and six courses of chemotherapy comprising intravenous bleomycin 30 IU, vinblastine 10 mg, doxorubicin 50 mg was planned. She received only two courses of chemotherapy due to fund constraints, and eight fractions of EBR before the equipment broke down. She was referred to continue treatment in a tertiary facility in another state (the only center in the country with a functional radiotherapy machine at the time) but was unable to do so on account of funds and logistical constraints. She visited the clinic a year after, and on examination, the left orbital tumor was slightly more than pretreatment size, with fungation. The patient was still constitutionally stable, and a fourth retroviral test was negative. She has been formally recommended to social services and other nongovernmental philanthropic bodies to facilitate her treatment.
|Figure 3 Picture of histology slide showing vasoformative lesion composed of sheets of fusiform cells with spindle-shaped nuclei typical of Kaposi sarcoma|
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| Discussion and Literature Review|| |
KS is a rare malignant tumor. In Jos, Nigeria, in a review of 4757 cases of histologically confirmed malignant tumors seen over a 16-year period, only 65 (1.37%) were KS, and although eight (12.3%) were located in the face, it was not stated if the eye was included as a facial location. The same review showed KS was four times more common in males than females and the most common location was the lower limbs. There are four variants of KS: classical, African (endemic), immunosuppression (transplant)-associated and AIDS-associated KS. The classic variant is seen commonly in HIV-negative patients and also does not have an aggressive natural history, whereas the African and AIDS-KS are more aggressive with frequent involvement of mucous membranes and viscera.,, Classical KS typically occurs in white elderly males often of Mediterranean origin, whereas African and AIDS associated types tend to occur in younger individuals including children.,
A study of classical KS in Morocco reports a mean age incidence of 61.7 years while in Calabar, Nigeria, the mean age for patients with AIDS-KS was 35.6 years and for the HIV-negative patients with KS it was 45 years., Our patient was not in the young age group but older, she is female and tested negative to HIV 1 and 2 antibodies on repeated testing. She also did not show evidence of visceral involvement and had no history of diabetes mellitus or use of immunosuppressive therapy. As typical of the classical variant, her disease ran an indolent course but was found in an unusual location, the orbit. There is paucity of reports of classical KS among black Africans; however, one case has been reported in a Nigerian farmer, but the location of the lesion was the lower limb.
Risk factors associated with classic KS include corticosteroid use, trauma, infrequent bathing, asthma, and a history of allergy in men., Our index patient had a preceding history of trauma which has been reported in literature to precipitate inflammatory changes that recruit HHV-8 to the injured site. HHV-8 is associated with the occurrence of KS, and in a review in Africans, the prevalence of HHV-8 was reported to be quite substantial ranging from 18.1% to 70.6%; however, there were no facilities for identification of HHV-8 in the biopsy specimen in or near the hospital, so this etiology could not be established.
The patient had a wedge incisional biopsy with marked intraoperative hemorrhage and intermittent postoperative bleeding from the tumor. Trucut core biopsy which has a high effectiveness of about 94% to 98% accuracy in diagnosis of specimens may have proved useful in this patient with a vascular tumor (KS), as it induces less trauma to the tissue and less hemorrhage.
Although there is no definitive cure for KS, reports have shown limited success with chemotherapy and excellent response to radiotherapy.,, The tendency toward multimodality makes radiation therapy or chemotherapy, or both, the preferred mode of treatment., Due to the huge size of the tumor, our patient was offered combined therapy comprising of an initial chemotherapy (six cycles) and radiotherapy (40-Gy EBR in 20 fractions) over 4 weeks, but she was unable to effectively take up treatment due to financial and logistical constraints as well as health-system failure.
| Conclusion and Recommendations|| |
This is a case of classical indolent KS with a rare occurrence in the orbit in a retroviral negative female. Costs and logistical challenges of accessing poorly available treatments, especially radiotherapy, were the problems encountered by this patient. These challenges can largely be resolved in a better funded health system providing needed equipment and infrastructure for a teeming population and health insurance coverage for all.
Declaration of patient consent
The authors certify that they have formally obtained the appropriate patient consent form. In the form, the patient gave her consent for her images and other clinical information to be reported in the journal. The patient understands that her names and initials will not be published and due efforts will be made to conceal her identity, but that anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]