Kaposis Sarcoma Is A Rare Disease Biology Essay
Kaposis sarcoma belongs among endothelial tumours with a particular local aggressiveness. It appears in the signifier of multiple skin lesions with different facets: spots, plaques or nodules. The tumours can besides happen in the mucous membrane, lymph nodes or splanchnic variety meats. The disease is associated with human herpes virus 8 ( HHV-8 ) .
Clinically and epidemiologically there are four types of Kaposi ‘s sarcoma:
– The classical painless type occurs chiefly in work forces, aged 50-70 old ages, in the Mediterranean and Eastern Europe. 1 The incidence in the general population is 0.02 % . 6 It appears as spots, plaques or nodules of reddish, purple or dark brown, which may ulcerate, normally in the distal appendages. These skin lesions are normally painless and may be associated with tumours of the haematopoietic system. 1, 5 They becomes painful when ulcerate or go septic. 3 More than 30 % of patients who develop authoritative signifier of Kaposi ‘s sarcoma will develop, in clip, 2nd primary malignance, particularly nonHodgkin malignant lymphoma. 5
– African endemic signifier, which occurs in grownups of 35-40 old ages, males and females in equal proportions, and Equatorial African kids, which are non infected with HIV. Sarcoma can be localized to the tegument and frequently has a long development. 3, 5 It has an incidence between 3-9 % . 6
– Iatrogenic signifier normally occurs in organ transplant patients who are having immunosuppressive intervention and in patients on corticosteroid therapy for assorted diseases. The neoplasm appears in a few months or old ages after organ organ transplant or immunosuppressive therapy. 3 The disease is 300 times less common in immunosuppressed patients fixing for a graft than in patients with AIDS. 6
– Kaposi sarcoma associated with human immunodeficiency syndrome ( HIV ) , which is the most aggressive signifier of the disease, normally occurs in patients who carry HIV-1, frequently dispersed among homo- and bisexual work forces and among adult females who have as spouses, bisexual work forces. The age scope in which appears the disease is 20-54 old ages. 5 It is the most aggressive type of sarcoma. 1 The malignant neoplastic disease appears when the CD4 count is less than 50/mm ? , with a prevalence of 15-34 % . 3, 6
The infective agent of Kaposi sarcoma has been sought for a long clip, until 1994, when Chang et Al highlight the association of sarcoma with human herpes virus ( HHV-8 ) . Kaposi sarcoma diagnosing is made by biopsy of skin lesions or affected variety meats. 1.3
The disease occurs most normally in the tegument, although were found a batch of instances in which were affected mucose membranes ( unwritten is most frequently affected, lesions happening in difficult and soft roof of the mouth, gums, lips ) , lymph nodes, entrails ( lungs, GI piece of land ) , without detecting skin lesions. In exceeding instances, the disease can impact the musculuss, encephalon, kidneys. 1, 3
NonHodgkin malignant lymphoma is responsible for approximately 5 % of all malignant tumours, ranking the 2nd topographic point between the malignances of the caput and cervix after the squamous cell carcinoma. ( 1, 2 )
Head and neck part is the most common topographic point of extranodal nonHodgkin malignant lymphoma, stand foring 10-20 % of all instances of nonHodgkin lymphoma. Typically, these lymphomas happening in the Waldeyer ring, preponderantly appear in male patients, except thyroid lymphomas which occur preponderantly in adult females. 4
In the unwritten pit, they occur about 10 % of all nonHodgkin malignant lymphomas of the caput and cervix. 4 In 70 % of instances, nonHodgkin lymphoma of the unwritten pit and oropharynx develops in the palatine amygdaloid nucleus, followed at a great distance from the soft roof of the mouth, gums, and lingua ( 3 ) . The mean age of patients developing lymphatic malignant neoplastic disease in the unwritten pit is 60 old ages. 4
Multiple malignant neoplastic diseases are more common entities in medical pattern in recent decennaries. Combinations of two or more malignant neoplastic diseases in the same patient at the same time or at some distance, occur more often. NonHodgkin malignant lymphoma, with nodal or extranodal finding, may be associated with assorted malignant neoplastic diseases. One of these combinations of two malignant neoplastic diseases is the association of nonHodgkin malignant lymphoma and Kaposi sarcoma.
We present a clinical instance of a 54 old ages old male patient, who comes in ENT exigency room of ColE›ea Hospital for the visual aspect of two tumours, one on the upper ridge, in forepart of all four incisives and the 2nd one, in the head covering roof of the mouth on the left side. This 2nd tumour causes dysphagia and respiratory hurt, sometimes facet of dyspnoea. What has struck at the sight of this patient was his physical visual aspect, the patient enduring from the Madelung disease about 18 old ages. ( Fig. 1, Fig. 2 )
From personal pathological history of the patient, we find that the patient has chronic hepatitis with B virus and suffered two surgeries, one for a left inguinal hernia, in 1962 and the 2nd an orthopaedic surgery for right femoral break due to a route accident in 2002. The patient is inveterate moderate consuming intoxicant and ex-smoker.
ENT clinical scrutiny and cranial nervousnesss reveal:
– Inspection – giants tumours on the both sides of the cervix, submandibular, occipital, between the shoulder blades, with the presence of multiple tegument tumours, ruddy, irregular, somewhat uneven ; ( Fig. 3 )
– Palpation – giants tumours on the both sides of the cervix, submandibular, occipital, soft to the touch, painless, with a lipomatosis consistence ;
– Examination of the unwritten pit – one tumour on the upper ridge, in forepart of all four upper incisives, mensurating about 3 inches, with nodular facet, guerrilla, milky, with haemorrhagic countries on the surface ; the other tumour is situated in the soft roof of the mouth on the left, mensurating about 3 inches, round-oval, dark brown, with big nidation base in the soft roof of the mouth. ( Fig. 4, Fig. 5 )
Clinical scrutiny shows some tumours in the upper limbs, chiefly from the bilateral deltoid, thoracic and scapular, lipomatosis facet, soft touch, Mobile on the deep and superficial programs. On the tegument of upper limb, thorax, venters and back are observed a batch of tumours with macular facet, reddish, irregular and unorganised. The staying physical scrutiny is normal. ( Fig. 6 )
Based on history and clinical scrutiny, the suspected diagnosing is tumour of the alveolar ridge, tumour of the roof of the mouth head covering and elephantine Madelung disease.
To corroborate the diagnosing we have to carry on a series of research lab and paraclinical probes.
Chest X ray reveals symphysis of the bilateral costo-phrenic fistulas, without pleuro-pulmonary metastases.
Blood trials are normal.
Based on history, clinical scrutiny ENT, general clinical scrutiny, laboratory geographic expedition and paraclinical probes, the positive diagnosing is tumour of the alveolar ridge, tumour of the roof of the mouth head covering and elephantine Madelung disease.
To corroborate the diagnosing we had removed several clinical entities that may look in the alveolar ridge, soft roof of the mouth and tegument.
Differential diagnosing of the tegument ‘s tumours is made with: hobnail hemangioma, hemangioma microvenular, spindle cell hemangioma, reactive angioendotheliomatosis, cutaneal angiosarcoma, pyogenic granuloma, bacillary angiomatosis, purple, Kaposi sarcomas, skin lesions in the AIDS disease. 2, 3
Sing the tumours of the unwritten pit, the differential diagnosing should be done, particularly with a series of tumour, both benign and malignant. The soft roof of the mouth may happen villoma, haemangioma, limfangiomas, fibromas, adipose tumor, adenomas, all belonging among benign. Of malignant tumours that may happen at this degree include: squamous cell carcinoma, basal cell carcinomas, but besides, nonHodgkin malignant lymphoma, Kaposi ‘s sarcoma, malignant melanoma, and bacillary infections, such as TB, pox, human immunodeficiency syndrome findings ( AIDS ) .
The ridge formations which are at issue in the differential diagnosing are the above lesions and in add-on bone tumours can happen, both benign and malignant.
Once we established the positive diagnosing, the operator decides for the first clip, surgical intervention of tumours of the unwritten pit. We remove both tumours, of the upper ridge and of the head covering roof of the mouth. The tumours are sent to histopathology, macroscopically scrutiny being, for the tumour of the alveolar crest: tumour the 3/1, 5/2 inches, with nodular visual aspect, brown hemorrhage in subdivision and for the tumour of the soft roof of the mouth: two tumours, one with 2.5 / 2/1 inches and the 2nd with 1/1/0, 5 inches, with nodular visual aspect, brown hemorrhage on the subdivision. Microscopically, the diagnosing is: Kaposi sarcoma for all the tumours, with verification by immunohistochemical scrutiny.
Due to the malignant character of the tumours and to the physical visual aspect of the patient, elephantine adipose tumor of the anterior cervical part and of all around the cervix, that disturb bilateral lung map and prevent rapid attack to the windpipe in instance of exigency tracheostomy, we decide to take the extra fat from that degree. Intervention takes topographic point five yearss after remotion of malignant tumours, while the patient was tested for possible HIV infection. The HIV trial come out negative. In the 2nd surgery, was removed as extra fat, weighing about 1,500 gms, and extra tegument adjacent to the tumours with skin ruddy tumours and some latero-cervical lymph of the right side. Pathological consequence was slightly surprising in footings of latero-cervical lymph nature. Therefore, macroscopically scrutiny shows: adipose tumor of 19/13/11 inches ( 1.53 kilogram ) , which incorporates a salivary secretory organ of 4/3/2 inches, piece of tegument at 25/3 inches, three lymph nodes with diameters 3 inches, 1 inches and 0.5 inches. Microscopically scrutiny confirms the formation of elephantine lipomatosis nature and salivary secretory organ tumour unchanged, cuticular formations are likely cancerous, Kaposi sarcoma ; in contrast, in the 3 inches latero-cervical lymph nodule, found cells of nonHodgkin malignant lymphoma type B. Both pieces of the first operation and those in the 2nd are sent to immunohistochemical scrutiny, corroborating histopathological diagnosing.
The patient is sent to the haematology clinic ColA?ea Hospital and Department of Oncology, in order to find appropriate intervention for both malignant agony. Besides met a multidisciplinary squad consisting of oncologist, haematologist physician, ENT physician, with whom were associated anesthetist and heart specialist to set up curative program must follow the patient. The understanding was established to optimise the curative consequence of chemotherapy intervention and that intervention benefit is increased, the patient should take as much of the extra fat from the latero-cervical and posterior cervical portion. This intercession was necessary in order to see the hereafter development of lymphoma nonHodgkin through early sensing of any perennial ganglion, which would non hold been possible if the fatty tissue in the cervix would hold remained in topographic point. Therefore, one month after the last surgery, we reoperate the patient and take big sums of fat, approximately 3.5 kilograms. ( Fig. 7, Fig. 8 )
The postoperative development was favourable, the patient was closely monitored in the surgical lesion, basal metamorphosis and acid-base, blood and bosom map.
After mending surgical, patient is treated with Bleomycin, Vincristine and FamorubicinA? for six months.
Six months after surgery, skin lesions disappeared wholly, wounds latero-cervical, anterior and posterior cervical cicatrix were normal, the patient ‘s general status is good, the back uping better intervention of hematologic chemotherapy.
Particularities of the instance
The multiple malignant neoplastic diseases of the ENT country are non rare, but when we are covering with a rare tumor such as Kaposi sarcoma, the surprise find of a 2nd malignant neoplastic disease, synchronal with the first, is even greater. Although the association of nonHodgkin malignant lymphoma with Kaposi sarcoma is the most common combination of multiple malignant neoplastic diseases in patients with sarcoma, most times the two malignant neoplastic diseases are metacrone, seldom coincident.
You besides need to retrieve that patient come to the physician due to the visual aspect of the tumours caused by Kaposi sarcoma, in the oral cavity, without being able to uncover, because physical visual aspect, the adenopathy developed because of haematological disease.
Another characteristic of the instance is that the patient is non infected with HIV, which means that he endure of classical Kaposi sarcoma, which is the more rare in HIV-negative to HIV-positive.
What should non be overlooked is the physical visual aspect of the patient, he really enduring three coincident tumours, one benign ( generalized lipomatosis- Madelung disease ) and two malignances ( Kaposi sarcoma and nonHodgkin malignant lymphoma ) .