Tumors

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Epidemiology

Lymphangiomas belong to the family of angioblastic tumors, which account for approximately 20% of mesenchymal tumors. Their frequency is significantly lower than haemangiomas and they have a higher incidence in childhood (90% of them are detected by the end of the first year of life), while the majority (approximately 70%) of them are already present at birth.

Lymphangiosarcoma is a rare, undifferentiated, highly malignant angioblastic tumor, that mostly develops in extremities affected by chronic lymphedema. It was first described in 1948 by Stewart and Treves (Stewart-Treves Syndrome), who reported their preliminary experience with 6 cases of lymphangiosarcoma developed in patients with post-mastectomy lymphedema, after a period from 6 months to 24 years since surgery. However, this is a very rare event, with an incidence of approximately 1% of cases. Experiences reported in the literature refer to over 130 cases of fulminant sarcomatosis who, except for a few, died within two years. This tumor, is not just related to secondary lymphedemas, but can also develop on primary ones; it can be located not only in the upper extremities, but also in the lower extremities, and can affect also males.

As to lymphomas, their overall incidence in the world population is not known. The incidence of lymphomas in Italy is about 4-5000 cases/year. In the US, non-Hodgkin’s lymphomas (NHL) rank seventh among the causes of cancer-related deaths. In Italy, mortality is approx. 5 cases per 100,000 inhabitants/year. Conversely, for Hodgkin’s Lymphoma (HL), mortality has remarkably dropped over the last 20 years, following new therapeutic protocols. The incidence of HL is of around 20% of all lymphomas. Lymphomas have been reported in patients of all ages, of both sexes, although, if considered globally, they prevail in middle aged/elderly individuals, and in males.

If compared to primary tumors of the lymphatic system, secondary tumors are very frequent. The frequency of secondary lymphatic tumors in carcinomas in different sites is 69%, on average. Higher percentage values are reported in lung cancer (93%), gastric cancer (89%), breast cancer (80%), and in epidermal tumors of the head and neck (80%).

Pathogenesis

Lymphangiomas belong to differentiated angioblastic tumors; however, their exact nature is still controversial, namely whether they are neoplasms proper, or tumor-like malformations, such as hamartomas. In the majority of cases, they are due to a primary, congenital dysfunction of peripheral lymphatic vessels, therefore they are based on congenital malformations, with either early or late onset. Cystic lymphangiomas develop in well-defined territories (neck, oral pavement, mediastinum, armpits, groin, retroperitoneum), and in close relation with the sites of embryonal lymphatic sacks. These paired formations have been detected in relation with the two internal jugular veins (jugular sac), with the external jugular vein and the subclavian vein (subclavian sac), internal iliac veins (posterior lymphatic sac). A smaller, odd and median lymphatic sac, (retroperitoneal sac) is located in the retroperitoneal space. Later in embryo life, lymphatic sacs are absorbed and disappear. Cystic lymphangiomas grow out of primary lymphatic sacs that have failed to fully regress and be reabsorbed.

The pathogenesis of lymphangiosarcoma is still unclear, although many reports seem to confirm the angiogenetic potentials of the lymphatic endothelium, which would allegedly be particularly enhanced by chronic, inveterate lymph flow stasis. Further, some specific growth factors purportedly exist, which, through local build-up promoted by lymph flow stasis, are likely to become a key pathogenetic trigger of cell degeneration.

As for lymphomas, today, Hodgkin’s lymphoma is considered to be a malignant tumor proper, due to its inexorable progression (if not properly treated) and the morphological and biological characteristics of H-RS (Hodgkin’s Reed-Sternberg) cells, which are its most prominent expression. Neoplastic attributes of H-RS cells are: proliferation capacity, aneuploidy, clonal nature, etc.. These cells may originate out of different lymphoid populations, mostly related to the T-line and, more rarely, to the B-line. They are capable of producing various types of cytokines and factors (interleukins, activation antigens, adhesion molecules, growth factors, etc.). Since cell activation, proliferation, and differentiation are known to be controlled mostly by intercellular signals, and since cytokines are coordination factors of immunological and inflammation responses, HL can be considered as a neoplastic process promoting an inflammation-like immunologic reaction.

In the pathogenesis of NHL, a small group of these tumors derives from cells of the central lymphoid compartment (lymphoid pool not yet exposed to antigens). However, in the majority of cases, NHLs arise from mature cells of the peripheral lymphoid compartment. Most commonly, the process is triggered in the lymph nodes, but the incidence of NHL onset in the spleen and in other areas outside the lymph nodes, especially if they are rich in lymphatic structures (MALT, mucosa-associated lymphoid tissue), is remarkably high. B cell NHLs are a highly heterogeneous group, both biologically and clinically. They may originate in one of the structures forming secondary follicles (germinal centre, mantle zone, marginal zone) of lymph nodes and the spleen, as well as in lymphoid areas associated with mucosae. Many T cell NHLs primarily originate from the paracortical area of somatic lymph nodes, sometimes from the spleen, and also from other lymphoid structures outside the lymph nodes.

With regard to secondary or metastatic tumors, tumors originating from epithelial tissues spread through the lymphatics into loco-regional tributary lymph nodes, whereas distant metastases of connective tissue tumors are most commonly spread via the blood circulation. Lymph node invasion takes place in various steps: minuscule fragments of cell clones come off the tumor edge and, taking up the form of a neoplastic embolus, flow across lymphatic spaces and reach lymphatic pre-collectors and collectors afferent to satellite lymph nodes. Lymphatic propagation routes of many tumors are generally foreseeable, based on the anatomic-topographic knowledge of the loco-regional lymphatic network. However, due to neoplastic obstruction of the lymphatics and lymph nodes, metastases may also follow unexpected routes (retrograde lymphatic embolism).

Phatologic anatomy classification and staging

Lymphangiomas are classified into simple, cavernous, and cystic. Simple (or capillary) lymphangiomas are mostly located in the subcutaneous tissue of the face, neck, the axillae, in areas corresponding to embryonal fissures, and often in internal organs. Their structure consists of a network of endothelium-coated capillaries, with no blood components inside. This is the main histological difference to hemangiomas, although it cannot be deemed to be 100% sure, since in some hemangiomas, capillary lumens can be without blood cells. Lymphoid cell build-ups separating capillary lumens are a more typical, but much less constant finding. However, today, with modern immunohistochemical techniques, lymphatic endothelium can be distinguished from blood endothelium (using D2-40, a monoclonal antibody which exclusively labels the endothelium of small lymphatic vessels– E. Fulcheri 2006). Cavernous lymphangiomas, apart from the skin and subcutaneous tissues, can also affect the mouth mucosae, the lips and tongue, resulting into macrochilia and macroglossia. They are most commonly localized in the cervical and axillary regions, more rarely in the extremities, eyelids, groin and internal organs (lungs, intestine, retroperitoneum, etc.). They consist of cavernous endothelium-coated spaces, either empty or filled with lymph, with thick connective walls, which could also include muscle elements. Cystic lymphangiomas (or cystic hygromas) are almost always located in the neck and the axillary regions. Mesenteric and retroperitoneal locations are more rare. They can become bulky tumors (up to more than 15 cm in diameter). The tumor has a spongy look when it consists of a multilocular structure, but it can also resemble a single big cyst. Hystologically, cysts are covered with flat endothelium and are separated by a thin fibrous mesh. Mesenteric lymphangiomas contain a milky fluid similar to chyle (chylous lymphangiomas). Lymphangioma circumscriptum is a particular variety of lymphangioma: it affects the skin, may vary in size, and also involve subcutaneous tissues. It is characterized by several and dilated lymphatic vessels, dermal papillae underneath a skin with varying degrees of acanthosis and hyperkeratosis. These lesions may develop on healthy skin or on lymphedematous limbs, they may look warty, and ulcers and lymphorrhagia may also be present.

Macroscopically, lymphangiosarcomas feature multiple nodular, coin-shaped blooms of a dark blue-purple color, which in some cases may acquire a map-like appearance and are often full of pus and haemorrhagic content. From a hystopathological point of view, vascular structures have been observed with spindle-shaped endothelial cells grouped into strands and partially reticular structures, often with intermediate spaces coated by single or multiple layers of endothelium, with pericytes and fibroblasts. In areas presenting more compact cells, polymorphous nuclei were observed with mitotic anomalies; in some areas, sarcomatous stroma consisted of lymphatic sinuses and blood pools.

As for lymphomas, an essential feature of the morphological picture of Hodgkin’s lymphoma is the total disruption of the healthy lymph node structure, due to a granuloma-like process, with the presence of a neoplastic (H-RS cells) and a reactive cell component (lymphocytes, plasma cells, granulocytes, macrophages, fibroblasts), that are closely mixed together. HL can be classified into 5 types: lymphocyte presominant; nodular sclerosis; mixed cellularity; lymphocyte depletion; lymphocyte-rich. It is difficult to define a histopathological picture of non-Hodgkin’s lymphoma, because of its great number of different histocytological pictures, many different clinical expressions, different disease progression and prognosis patterns. The REAL (Revised European-American Classification of Lymphomas) classification is an attempt at providing a critical interpretation of lymphomatous pictures based on all the wealth of information we have today about the lymphomatous process: B cells NHL, lymphomas deriving from precursor B cells (leukemia/ B-lymphoblastic lymphoma) and lymphomas deriving from peripheral B cells (leukaemia, B cells chronic lymphatic, prolymphocytic leukemia, small cell lymphocytic lymphoma, lymphoplasmacytoid lymphoma or immunocytoma, mantle cell lymphoma, follicular centre lymphomas, marginal zone B cell lymphoma, monocytoid B cell lymphoma, MALT-lymphomas, hairy cell leukemia, plasmacytoma, large B cell lymphoma, Burkitt’s lymphoma); T and NK cell NHL, lymphomas deriving from precursor T cells (leukemia/ T-lymphoblastic lymphoma) and lymphomas deriving from peripheral T cells (peripheral T cell lymphoma unspecified, chronic T cell lymphatic leukemia, prolymphocytic T leukemia, large granulated lymphocytes leukemia, fungoid mycosis and Sézary syndrome, hepatosplenic T gamma-delta cell lymphoma, subcutaneous panniculitic T cell lymphoma, angioimmunoblastic lymphoma, angiocentric lymphoma, T cell primary intestinal lymphoma, associated with enteropathy, T cell lymphoma/leukemia of the adult, large anaplastic cell lymphoma).

MALT-lymphomas are particularly interesting from a surgical point of view. They are tumors derived from mucosa-associated lymphoid tissue (MALT), which includes Gut-associated lymphoid tissue (GALT) and Bronchus-associated lymphoid tissue (BALT), both referred to organs with mucosae containing lymphoid structures. It was later observed that in the stomach - although lined with mucosae - and in other organs that have no mucosae, there is no MALT type tissue under healthy conditions, but it can develop following flogistic-reactive or autoimmune events. Therefore, a primary MALT and a secondary MALT (for example: Helicobacter pylori associated chronic follicular gastritis, Hashimoto thyroiditis, etc.) have been defined. MALT may have either a single or multi-centric origin in the same viscus. The presence of lymphoepithelial lesions by centrocyte-like (CCL) cells, which invade and destroy epithelial-glandular structures of the lymphoma hosting organ, is a key histopathological finding of low grade MALT-NHL. Low grade MALT-NHLs may progress to become highly malignant, with centroblastic and/or plasmablastic and/or anaplastic cells. 30-50% of primary lymphomas outside the lymph nodes begin in the gastrointestinal tract. They are almost exclusively NHLs. Primary lymphomas of the gastrointestinal tract are staged as follows: Stage I –involvement of the gastrointestinal tract only, no serosa involvement (single or multiple primary localization, non-contiguous localizations); Stage II – lymphoma extending from the primary gastrointestinal site to the lymph nodes (tributary regional– II1 – or disseminated abdominal– II2); Stage III – the tumor extends beyond the serosa and infiltrates adjacent organs and/or structures; Stage IV – disseminated involvement outside the lymph nodes or involvement of supra-diaphragmatic lymph nodes.

In secondary tumors, marginal sinuses into which afferent lymphatics flow, are the initial sites of metastases. They then spread to the entire lymph node, while the capsule can still remain healthy. From here, they spread from lymph node to lymph node and, through extracapsular invasion, affected lymph nodes become attached to each other while the tumor trespasses into surrounding tissues. Tumoral cell clones can be released from the secondary lymphatic tumor, and, through efferent lymphatics, they can colonize other lymph nodes and invade the blood stream (in particular, through the thoracic duct on the left supraclavicular side).

Physiopathology and clinical aspects

A simple lymphangioma is difficult to distinguish from lymphangectasia, by looking at both clinical and histopathological features. It is a protrusion that can have different sizes, is whitish in color, with a soft, easy to press consistency. The subcutaneous tissue of the face and neck is the most common site. Simple lymphangiomas are slow growing tumors, and in most cases do not cause any severe problems, apart from aesthetics. At physical examination, cavernous lymphangiomas look like bluish-reddish areas, interspersed with translucent, thicker areas, as well as areas that can be pressed under the touch. The tongue (macroglossia) and the lips (macrocheilia) are typical sites. Apart from visible deformities, cavernous lymphangiomas in an oral site may cause problems in speaking, swallowing, and breathing. Major complications are cutaneous or mucosal ulcers with subsequent haemorrhages and/or lymphorrhagia, as well as recurrent lymphangitis. Cystic lymphangioma prevailingly occurs in the neck, rarely does it extend to the mediastinum, the armpits, or the tongue. In the majority of cases, only few symptoms are reported. However, the opisthotonic posture of the affected child may cause problems with feeding and breathing. When involving the tongue, the mouth pavement, and/or the mediastinum, dysphagia and dyspnea may also be reported. Severe respiratory insufficiency is more common with rare cases of cervical-mediastinal sandglass-like cystic lymphangiomas. In case of respiratory problems or haemorrhages linked to the tumor, a sudden and significant growth in lymphangioma volume can also occur. Infection is a rare but dangerous complication, and a tumor rupture may also cause a chylothorax.

The clinical progression and individual growth stages of Stewart-Treves lymphangiosarcoma are quite uniform. The disease affects a lymphedematous limb, with spontaneous formation of painful and tender bruises or haematomas, bluish nodules, skin plaques, papules, and ulcerations. Progression and histopathology of the Stewart-Treves syndrome are very similar to those described for the first time by Kaposi in 1872, for what he, in 1894, called sarcoma idiopaticum multiplex haemorragicum. Ackermann and Murray (1963) demonstrated that, actually, they are two quite discreet entities. Prognosis makes the difference: in Kaposi’s sarcoma, many years, even decades, my pass from the initial blooms and the growth of nodules, whereas in Stewart-Treves lymphangiosarcoma, the patient generally dies within two years. Kaposi’s sarcoma hits mostly males, whereas S.-T. lymphangiosarcoma prevailingly affects women (linked to post-mastectomy lymphedema). Also, Kaposi’s sarcoma is normally associated with AIDS, and, if so, it has a poorer prognosis.

The onset of Hodgkin’s lymphoma presents a clinical picture that varies from case to case. Indeed, the disease may present itself with different stages of spread, in different localizations, and with or without general symptoms: mild fever, or high and irregular rises in body temperature, sweating during the night (especially on the back of the neck), diffuse or localized itching, severe physical weakness, weight loss. Early appearance of surface and/or deep lymphoadenomegalies. Among surface localizations, lateral-cervical and supraclavicular ones are the most common (and, in particular, on the left side of the neck). Superficial lymph nodes vary in size, generally their volume is increased, they are hard with a smooth surface, indolent (another rarer feature is that they are particularly painful to the touch after alcohol intake), mobile and initially discreet, later to join into clusters and adhere to the surrounding tissues. Mediastinal localization may cause a mediastinal syndrome, due to the involvement of vascular and nervous, aerial, and digestive structures of the mediastinum. Abdominal lymph node localizations are often clinically silent, although they may cause some generic digestion problems. In some rare cases, a peripheral lymphatic-venous stasis in the lower limbs and external genitalia may occur.

With regard to non-Hodgkin’s lymphoma, whatever forms it takes, it always has an insidious onset: sometimes it is characterized by a progressive symmetrical swelling of superficial lymph nodes in supra- and sub-diaphragmatic sites, in other cases (10%) an apparently isolated splenomegaly, or a slowly progressing anaemia, or the presence of skin nodules can be observed. Superficial lymph nodes are symmetrically arranged, generally small in size, mobile, not painful, with a smooth surface and parenchymatous consistency; there are no adhesions to the skin, nor do they form clusters, at least not until later stages. Some large abdominal lymph node masses can be present merging together in the retroperitoneum and the mesentery. Waldeyer’s tonsillar ring and the tonsils may be hypertrophic, causing problems during swallowing and phonation. The spleen is generally enlarged, in some cases also significantly so. Hepatomegaly is a rare finding, just like jaundice, caused by compression of the biliary ducts by the lymph nodes in the hepatic hilus.

As to clinical assessment of nodules of likely malignant neoplastic origin (e.g. thyroid, breasts, skin, etc.), a careful examination of satellite lymph nodes by the touch (in the cervical, supraclavicular, axillary, and inguinal region) is important, in order to detect any lymphoadenomegalies that may be related to secondary regional lymph node localization of tumor metastases.

Diagnosis

With regard to lymphangiomas, diagnostic suspicion can be raised on the basis of the above described clinical features. An accurate diagnosis should be supported by ultrasounds, CT scan and MRI, which can provide information about the extent of disease as well as about its connections with neighboring structures. Lymphoscintigraphy can highlight the presence of correlations between lymphangioma and all major lymphatic pathways draining the area concerned, in particular localizations in the extremities. This is key information for preventive diagnosis on the likely onset of secondary lymphedema after tumor resection.

In lymphangiosarcoma, a biopsy of skin nodules with the above described clinical features, and confirmed by CT scan and MRI, is particularly useful. In this case too, lymphoscintigraphy can provide useful information on any damage to the superficial as well deep lymphatic drainage system of the affected limb/s, also considering that in the majority of cases lymphangiosarcoma develops in already lymphedematous extremities.

Recommended investigations to diagnose a Hodgkin’s or non-Hodgkin’s lymphoma and determine its extension (staging) include the following: clinical examinations and lab tests (medical history, physical examination, routine lab and optional tests, such as Ig essay, serum immunoelectrophoresis, etc., immunological monitoring); Imaging (Chest x-Rays, abdominal ultrasound and/or of superficial organs, such as lymph nodes, thyroid, etc., CT scan of the chest-abdomen-pelvis, MRI and other optional examinations, e.g. alimentary tract, PET, lymphography, etc.); Routine invasive examinations (surgical lymph node biopsy, bone marrow aspirations and spinal puncture biopsy, and cytological examination of cerebrospinal fluid); Optional invasive examinations (laparotomy or laparoscopy with splenectomy and multiple biopsies, tapping of pleural, pericardial, and abdominal effusions, cytological examination, digestive tract endoscopy).

Finally, with regard to the detection of secondary lymph node tumors, particularly ultrasonography and CT scan have proved to be most useful, because they allow for an accurate primary tumor staging, not only with regard to the size and local extension of the lesion, (T), but also to lymph node (N) and distant (M) metastases.

Therapy

In small cystic asymptomatic lymphangiomas, there are no indications for immediate surgery. For an easier surgery, it is advisable to postpone it to a more adult age. The only indications for emergency surgery are suppurative complications - in which case an incision is necessary for drainage - and acute respiratory failure, for which a tracheotomy must be performed and/or a suction drainage placed under strictly aseptic conditions. Sclerosing therapy for neck lymphangioma is not advisable, due to the risk of vascular or nervous lesions. The only effective therapy is surgical resection, which is seldom radical, however, it must be recalled that cystic lymphangiomas, and especially when they are extended, rarely have clearcut margins, but they rather infiltrate into surrounding structures, hence there is the risk of damaging major nervous structures (7th , 9th , 12th pair of cranial nerves). For these reasons, recurrencies are not uncommon and repeated surgeries, at more or less close intervals, must be performed.

In the case of lymphangiosarcoma, surgical resection of nodules is often only palliative, although long-lasting disease-free cases have been reported after resection of individual nodules. However, early diagnosis of lymphangiosarcoma is the most important preventive and therapeutic, as well as prognostic factor. In case of a single nodules, which still looks histologically benign - namely that is not yet invasive or infiltrating -, an extensive surgical resection of the nodule must be followed by radiation therapy and an accurate follow-up. Conversely, when the histopathological picture is overtly malignant, with such a level of dissemination that a fulminating tumor growth is most likely to occur, amputation with limb disarticulation is the only treatment. Indeed, chemotherapy, both loco-regional (hyperthermal isolated limb perfusion) and systemic (interleukine-2 immunotherapy), with an average survival rate of 18 months, has failed to provide encouraging results.

With regard to lymphomas, apart from a few of them that are really impervious, if properly treated, they can be kept in check with a thorough and long-lasting follow-up. The following therapeutic tools are currently available: ionizing radiations, antiblastic cytostatic drugs, substances that can modulate the body response to the tumor (corticosteroids, interferon, etc.), marrow or peripheral stem cell transplantation, surgery, etc.. With regard to surgery, its role in the treatment of lymphomas concerns two aspects in particular: tumor staging (laparosplenectomy, in particular in Hodgkin’s lymphomas), and organ surgery (resection of tumor masses, more common in non-Hodgkin’s lymphomas). Surgical staging includes splenectomy, liver biopsy, resection of enlarged lymph nodes with the application of reference metal clips in each site for subsequent radiation therapy. Therefore, surgical staging is recommended when radiation is required, while it is deemed useless when the tumor is treated only with chemotherapy (stage I and II HL; stage I NHL). Splenectomy is indicated for all stages, when severe splenomegaly is present, but it is not recommended under 15 years of age (when there is the risk of fulminant post-splenectomy sepsis). Surgery plays a key role in extranodal lymphomas (organ tumor). The stomach is the most commonly affected organ, in particular in B-cell NHL. Distant survival is better than with carcinoma after aggressive surgery. The degree of radicality in this type of surgery has been reported to depend on tumor size, penetration and spread, rather than histotype. The survival rate in early stage (I and II) tumors is similar to early cancer (90-95% at 5 years). In more advanced (III and IV) stages, surgical resection can be taken into account in order to reduce the tumor and improve symptoms. Palliation has indeed yielded surprisingly good, even long-term results, due to the less aggressive nature of lymphoma than carcinoma. The small intestine, and the ileum in particular, is another, although rare, extranodal indication to surgery in NHL. The clinical picture at its onset generally features an occlusion or the development of a tender swelling, together with weight loss. The intestinal segment involved is resected, and a lymphadenectomy is performed at the corresponding level of the mesenteric fan. Radical surgery of a malignant epithelial tumor generally features a wide loco-regional lymph node resection. It has a certain curative function, if the tumor has been stopped at this level, without distant metastases. It also plays an important role for prognostic reasons, since it allows accurate staging of the tumor, hence to properly design the required integrated therapeutic strategy (e.g. chemotherapy, radiation therapy, etc.). In lymphangiomas, percutaneous sclerotherapy is currently playing a key therapeutic role, in particular in microcystic tissue forms and in cystic hygromas: good clinical results can be achieved with this non-invasive technique, even with almost complete disease regression of lymphatic vescicles or spaces. Depending on lesion size, the most suitable sclerosant is selected: in smaller forms, polidocanol is used, in more bulky cases with extended lymphatic spaces, ethanol or Ethibloc should be used. Sclerosant injection must be followed by a good selective loco-regional compression, especially in forms with extended lymphatic spaces.

Complementary and alternative therapies to surgery must be taken into account, in particular, in the treatment of lymphomas. Therapy in these cases basically features a combination of radiation and chemotherapy, associated with several adjuvant therapies: whole blood transfusions of concentrated erythrocytes or platelet-rich plasma, anabolic steroids, vitamins (A, C, folates), antiemetics, antibiotics, and antibacterial and antimicotic chemotherapic drugs, hematopoietic growth factors (erythropoietin), transplantation of marrow stem cells or of autologous, or allogenic peripheral stem cells, etc. The sensitivity of lymphomatous cells to radiation or cytostatic agents and the efficacy of antitumor therapies depend on many variables (biological features of lymphomatous cells, tumor extension and volume, etc.). Similarly, how well the patient’s body is able to tolerate treatment depends on his/her age, the extent of organ involvement by lymphoma, and the presence of other concurrent/previous diseases.

With regard to radical lymphadenectomy for oncological reasons, when performed in critical regions such as the armpits and the groin, for the treatment of epithelial tumors (breast, vulva, penis, melanoma, etc.), this surgical lymph node resection is not without early (e.g. lymphorrhoea, lymphangitis, suture dehiscence), as well as late (lymphedema) complications. For example, with reference to breast cancer, the incidence of secondary lymphedema of the upper limb in women who have undergone mastectomy or quadrantectomy with axillary lymphadenectomy ranges from 20-25%, to 35-40%, when associated with radiation therapy. Secondary lymphedema of the lower limbs following treatment of gynaecological or urological tumors has an incidence from 5% to 30%. Therefore, the importance of an alternative approach to radical lymphadenectomy - namely the sentinel lymph node - can be easily understood. Sentinel lymph node means the first lymph node receiving lymphatic drainage from the area of the primary tumor, which can thus be the site of early tumor metastases. With this method, a more rational surgical approach to regional lymph nodes is possible, while making surgery less invasive, thus significantly decreasing the incidence of the above mentioned complications. At the same time, it allows for an accurate histological and immunohistochemical assessment of the lymph nodes with the highest metastatic risk, while providing adequate tumor staging.

Figure 1, A-D: Cystic lymphangioma of the left lateral-cervical region.

Figure 2, A-B Kaposi’s Sarcoma of the right lower limb not correlated with HIV infection.