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Kaposis sarcoma belongs among endothelial tumors with a special local aggressiveness. It appears in the form of multiple skin lesions with different aspects: patches, plaques or nodules. The tumors can also occur in the mucosa, lymph nodes or visceral organs. 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 mainly in men, aged 50-70 years, in the Mediterranean and Eastern Europe. 1 The incidence in the general population is 0.02%. 6 It appears as patches, plaques or nodules of reddish, purplish or dark brown, which may ulcerate, usually in the distal extremities. These skin lesions are usually painless and may be associated with tumors of the hematopoietic system. 1, 5 They becomes painful when ulcerate or become infected. 3 More than 30% of patients who develop classic form of Kaposi's sarcoma will develop, in time, second primary malignancy, especially nonHodgkin malignant lymphoma. 5
- African endemic form, which occurs in adults of 35-40 years, males and females in equal proportions, and Equatorial African children, which are not infected with HIV. Sarcoma can be localized to the skin and often has a long evolution. 3, 5 It has an incidence between 3-9%. 6
- Iatrogenic form usually occurs in organ transplant patients who are receiving immunosuppressive treatment and in patients on corticosteroid therapy for various diseases. The neoplasm appears in a few months or years after organ transplantation or immunosuppressive therapy. 3 The disease is 300 times less common in immunosuppressed patients preparing for a transplant than in patients with AIDS. 6
- Kaposi sarcoma associated with human immunodeficiency syndrome (HIV), which is the most aggressive form of the disease, usually occurs in patients who carry HIV-1, often spread among homo- and bisexual men and among women who have as partners, bisexual men. The age range in which appears the disease is 20-54 years. 5 It is the most aggressive type of sarcoma. 1 The cancer appears when the CD4 count is less than 50/mm ³, with a prevalence of 15-34%. 3, 6
The infectious agent of Kaposi sarcoma has been sought for a long time, until 1994, when Chang et al highlight the association of sarcoma with human herpes virus (HHV-8). Kaposi sarcoma diagnosis is made by biopsy of skin lesions or affected organs. 1.3
The disease occurs most commonly in the skin, although were found a lot of cases in which were affected mucous membranes (oral is most often affected, lesions occurring in hard and soft palate, gums, lips), lymph nodes, viscera (lungs, gastrointestinal tract), without discovering skin lesions. In exceptional cases, the disease can affect the muscles, brain, kidneys. 1, 3
NonHodgkin malignant lymphoma is responsible for about 5% of all malignant tumors, ranking the second place between the malignancies of the head and neck after the squamous cell carcinoma. (1, 2)
Head and neck region is the most common place of extranodal nonHodgkin malignant lymphoma, representing 10-20% of all cases of nonHodgkin lymphoma. Typically, these lymphomas occurring in the Waldeyer ring, predominantly appear in male patients, except thyroid lymphomas which occur predominantly in women. 4
In the oral cavity, they occur about 10% of all nonHodgkin malignant lymphomas of the head and neck. 4 In 70% of cases, nonHodgkin lymphoma of the oral cavity and oropharynx develops in the palatine amygdala, followed at a great distance from the soft palate, gums, and tongue (3). The average age of patients developing lymphatic cancer in the oral cavity is 60 years. 4
Multiple cancers are more common entities in medical practice in recent decades. Combinations of two or more cancers in the same patient simultaneously or at some distance, occur more frequently. NonHodgkin malignant lymphoma, with nodal or extranodal determination, may be associated with various cancers. One of these combinations of two cancers is the association of nonHodgkin malignant lymphoma and Kaposi sarcoma.
We present a clinical case of a 54 years old male patient, who comes in ENT emergency room of ColÈ›ea Hospital for the appearance of two tumors, one on the upper ridge, in front of all four incisives and the second one, in the veil palate on the left side. This second tumor causes dysphagia and respiratory distress, sometimes aspect of dyspnea. What has struck at the sight of this patient was his physical appearance, the patient suffering from the Madelung disease about 18 years. (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 second an orthopedic surgery for right femoral fracture due to a road accident in 2002. The patient is chronically moderate consuming alcohol and ex-smoker.
ENT clinical examination and cranial nerves reveal:
- Inspection - giants tumors on the both sides of the neck, submandibular, occipital, between the shoulder blades, with the presence of multiple skin tumors, reddish, irregular, slightly uneven; ( Fig. 3)
- Palpation - giants tumors on the both sides of the neck, submandibular, occipital, soft to the touch, painless, with a lipomatosis consistency;
- Exam of the oral cavity - one tumor on the upper ridge, in front of all four upper incisives, measuring around 3 inches, with nodular aspect, irregular, whitish, with hemorrhagic areas on the surface; the other tumor is situated in the soft palate on the left, measuring around 3 inches, round-oval, dark brown, with large implantation base in the soft palate. (Fig. 4, Fig. 5)
Clinical examination shows some tumors in the upper limbs, mainly from the bilateral deltoid, pectoral and scapular, lipomatosis aspect, soft touch, mobile on the deep and superficial plans. On the skin of upper limb, thorax, abdomen and back are observed a lot of tumors with macular aspect, reddish, irregular and unorganized. The remaining physical examination is normal. (Fig. 6)
Based on history and clinical examination, the suspected diagnosis is tumor of the alveolar ridge, tumor of the palate veil and giant Madelung disease.
To confirm the diagnosis we have to conducted a series of laboratory and paraclinical investigations.
Chest X-ray reveals symphysis of the bilateral costo-phrenic sinuses, without pleuro-pulmonary metastases.
Blood tests are normal.
Based on history, clinical examination ENT, general clinical examination, laboratory exploration and paraclinical investigations, the positive diagnosis is tumor of the alveolar ridge, tumor of the palate veil and giant Madelung disease.
To confirm the diagnosis we had removed several clinical entities that may appear in the alveolar ridge, soft palate and skin.
Differential diagnosis of the skin's tumors is made with: hobnail haemangioma, haemangioma microvenular, spindle cell haemangioma, reactive angioendotheliomatosis, cutaneous angiosarcoma, pyogenic granuloma, bacillary angiomatosis, purple, Kaposi sarcomas, skin lesions in the AIDS disease. 2, 3
Regarding the tumors of the oral cavity, the differential diagnosis should be done, especially with a series of tumor, both benign and malignant. The soft palate may occur papillomas, hemangiomas, limfangiomas, fibromas, lipomas, adenomas, all belonging among benign. Of malignant tumors that may occur at this level include: squamous cell carcinoma, basal cell carcinomas, but also, nonHodgkin malignant lymphoma, Kaposi's sarcoma, malignant melanoma, and bacillary infections, such as tuberculosis, syphilis, human immunodeficiency syndrome determinations (AIDS).
The ridge formations which are at issue in the differential diagnosis are the above lesions and in addition bone tumors can occur, both benign and malignant.
Once we established the positive diagnosis, the operator decides for the first time, surgical treatment of tumors of the oral cavity. We remove both tumors, of the upper ridge and of the veil palate. The tumors are sent to histopathology, macroscopically examination being, for the tumor of the alveolar crest: tumor the 3/1, 5/2 inches, with nodular appearance, brown bleeding in section and for the tumor of the soft palate: two tumors, one with 2.5 / 2/1 inches and the second with 1/1/0, 5 inches, with nodular appearance, brown bleeding on the section. Microscopically, the diagnosis is: Kaposi sarcoma for all the tumors, with confirmation by immunohistochemical examination.
Due to the malignant character of the tumors and to the physical appearance of the patient, giant lipomas of the anterior cervical region and of all around the neck, that disturb bilateral lung function and prevent rapid approach to the trachea in case of emergency tracheotomy, we decide to remove the excess fat from that level. Intervention takes place five days after removal of malignant tumors, while the patient was tested for possible HIV infection. The HIV test come out negative. In the second surgery, was removed as excess fat, weighing about 1,500 grams, and excess skin adjacent to the tumors with skin reddish tumors and some latero-cervical lymph of the right side. Pathological result was somewhat surprising in terms of latero-cervical lymph nature. Thus, macroscopically examination shows: lipoma of 19/13/11 inches (1.53 kg), which incorporates a salivary gland of 4/3/2 inches, piece of skin at 25/3 inches, three lymph nodes with diameters 3 inches, 1 inches and 0.5 inches. Microscopically examination confirms the formation of giant lipomatosis nature and salivary gland tumor unchanged, dermal 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 second are sent to immunohistochemical examination, confirming histopathological diagnosis.
The patient is sent to the hematology clinic ColÅ£ea Hospital and Department of Oncology, in order to determine appropriate treatment for both malignant suffering. Also met a multidisciplinary team consisting of oncologist, hematologist doctor, ENT doctor, with whom were associated anesthesiologist and cardiologist to establish therapeutic plan must follow the patient. The agreement was established to optimize the therapeutic effect of chemotherapy treatment and that treatment benefit is increased, the patient should remove as much of the excess fat from the latero-cervical and posterior cervical part. This intervention was necessary in order to see the future development of lymphoma nonHodgkin through early detection of any recurrent ganglion, which would not have been possible if the fatty tissue in the neck would have remained in place. Thus, one month after the last surgery, we reoperate the patient and remove large amounts of fat, about 3.5 kg. (Fig. 7, Fig. 8)
The postoperative evolution was favorable, the patient was closely monitored in the surgical wound, basal metabolism and acid-base, blood and heart function.
After healing surgical, patient is treated with Bleomycin, Vincristine and FamorubicinÄƒ for six months.
Six months after surgery, skin lesions disappeared completely, wounds latero-cervical, anterior and posterior cervical scar were normal, the patient's general condition is good, the supporting better treatment of haematological chemotherapy.
Particularities of the case
The multiple cancers of the ENT area are not rare, but when we are dealing with a rare neoplasm such as Kaposi sarcoma, the surprise discovery of a second cancer, synchronous with the first, is even greater. Although the association of nonHodgkin malignant lymphoma with Kaposi sarcoma is the most common combination of multiple cancers in patients with sarcoma, most times the two cancers are metacrone, rarely simultaneous.
You also need to remember that patient come to the doctor due to the appearance of the tumors caused by Kaposi sarcoma, in the mouth, without being able to reveal, because physical appearance, the adenopathy developed because of hematologic disease.
Another feature of the case is that the patient is not infected with HIV, which means that he suffer of classical Kaposi sarcoma, which is the more rare in HIV-negative to HIV-positive.
What should not be overlooked is the physical appearance of the patient, he actually suffering three simultaneous tumors, one benign (generalized lipomatosis- Madelung disease) and two malignancies (Kaposi sarcoma and nonHodgkin malignant lymphoma).