TUMORS

TUMORS

EPIDEMIOLOGY

Lymphangiomas are part of angioblastic tumors, which account for about 20% of mesenchymal tumors. They have a significantly lower frequency compared to hemangiomas and have a greater incidence in childhood (90% are identified at the end of the first year of life) and most of them (about 70%) are already present at birth.

Lymphangiosarcoma is an undifferentiated, rare, extremely malignant angioblastic tumor that develops predominantly in a limb with chronic lymphedema. It was first described in 1948 by Stewart and Treves (Stewart-Treves syndrome), referring to a preliminary experience of 6 cases of lymphangiosarcoma, developed on post-mastectomy lymphedema, after a period of time ranging from 6 months to 24 years after the surgery. This tumor occurs very rarely, with an incidence of approximately 1% of cases. The experiences described in the literature concern over 130 cases of fulminant sarcomatosis which, except for a few, lead to death within two years. The tumor can also develop in patients with primary lymphedema and also in the lower limbs and in the male geneder.

As far as lymphomas are concerned, the real global incidence in the world population is unknown. The incidence of lymphomas in Italy is about 4-5,000 cases / year. In the US, non-Hodgkin’s lymphomas (NHL) are in 7th place among the causes of death due to malignant neoplasm. In Italy, mortality is about 5 cases per 100,000 inhabitants / year. Instead, for Hodgkin’s disease (MH), mortality has drastically decreased over the last 20 years, as a result of new therapeutic approaches. In addition, HD has an incidence that is approximately 20% of all lymphomas. Lymphomas have been observed in all ages of life and in both sexes, however, globally considered, they are more frequent in middle and advanced ages and in males.

Compared to the primary tumors of the lymphatic system, secondary tumors are very frequent. The frequency of secondary lymphatic tumors as carcinomas in various sites is on average 69%. The highest percentages are lung tumors (93%), stomach (89%), breast (80%) and epidermal head and neck tumors (80%).

PATHOGENESIS

Lymphangiomas are part of the differentiated angioblastic tumors and their exact nature is still controversial: as to whether they are true neoplasms or simil-tumoral malformations, such as hamartomas. In most cases, they are due to a primary anomaly of the peripheral lymphatic vessels and, therefore, are on a congenital malformative basis, as an early or late manifestation. Cystic lymphangiomas appear in well-defined areas (neck, oral floor, mediastinum, axilla, groin, retroperitoneum) in close relationship with the site of embryonic lymphatic sacs. These balanced tumor formations have been found in connection with the two internal jugulars (jugular sac), with the external jugular, and the subclavian (subclavian sac), and with the internal iliac veins (posterior lymphatic sac). A smaller, unequal and median lymphatic sac (retroperitoneal sac) is located in the retroperitoneal space. Lymphatic sacs are destined to disappear due to reabsorption. Cystic lymphangiomas arise from the lack of or incomplete regression and reabsorption of these primitive lymphatic sacs.

The pathogenesis of lymphangiosarcomas, to date, is not clear, but many studies seem to confirm the angiogenic potential of lymphatic endothelium, which would be particularly increased by inveterate chronic lymphatic stasis. Moreover, specific growth factors would exist which, accumulating locally due to lymph stasis, could represent the fundamental pathogenic motive of formation.

Regarding lymphomas, today it is believed that Hodgkin’s disease represents a real malignant neoplasia, for its inexorable clinical progression (if not adequately treated) and for the morphological and biological characteristics of H-RS cells (Hodgkin-Reed – Sternberg), which are the most salient expression of the process. The neoplastic attributes of H-RS cells are represented by: ability to proliferate, aneuploidy, clonal character, etc. These cells can be derived from different lymphoid populations, more often arising to the T-line and more rarely to the B. They have a marked ability to produce cytokines and various factors (interleukins, activation antigens, adhesion molecules, growth factors, etc.). Since it is known that cell activation, proliferation and differentiation are mostly controlled by intercellular signals, and that cytokines are coordinating factors of immunological and inflammatory responses, HD appears as a neoplastic process that gives rise to an inflmmatory reactive process.

 

In the pathogenesis of LNH, a small group derives from cells of the central lymphoid compartment (lymphoid pool not yet exposed to antigens) but, for the most part, the LNH derive from mature cells of the peripheral lymphoid compartment. With particular frequency the process starts in lymph nodes, but the incidence of onset in the spleen and in other extra-lymph node areas is also remarkable, especially if rich in lymphatic structures (MALT, lymphoid tissue associated with the mucosa). B-cell LNHs represent a group of high biological and clinical heterogeneity. The secondary follicles (germinal center, mantle area, marginal zone) of the lymph nodes and of the spleen and in lymphoid areas associated with the mucous membranes can originate in one of the component structures. Many T-cell LNHs originate primarily in the paracortical area of ​​somatic lymph nodes, sometimes in the spleen and also in extra-nodal lymphoid structures.

With regard to secondary or metastatic tumors, tumors derived from epithelial tissues spread lymphatically into the local-regional tributary lymph nodes, while the connective tissue tumors follow, in their remote diffusion, preferably the blood route. The lymphoglandular invasion takes place in stages, from the edge of the tumor tiny cellular clones are detached that, through the lymphatic lacunae in the form of neoplastic embolus, reach the precollectors and lymphatic collectors afferent to the satellite lymph node stations. The lymphatic propagation pathways of many tumors are, in general, predictable on the basis of anatomorphographic knowledge concerning the distribution of the loco-regional lymphatic network. However, due to lymphatic-lymph node neoplastic obstruction, metastasis can take completely abnormal pathways (retrograde lymphatic embolism).

 

ANATOMO-PATHOLOGICAL CLASSIFICATION AND STAGING

Lymphangiomas are distinguished into simple, cavernous, and cystic forms. The simple lymphangiomas (or capillary forms) are mainly located in the subcutaneous tissue of the face, neck and axilla, in areas corresponding to embryonic fissures, and, more rarely, in internal organs. Their structure is formed by a network of capillaries coated with endothelium that do not contain blood cells inside. This is the main data of histological differentiation from hemangiomas, even if it cannot be considered a datum of absolute certainty, since in some hemangiomas, the capillary lumens may be deprived of blood cells. It is more typical, but much less constant, the finding of accumulations of lymphoid cells in the connective tissue that separates the capillary lumens. However, thanks to modern immunohistochemical investigation techniques, it is now possible to distinguish the lymphatic endothelium from the hematic endothelium (D2-40, monoclonal antibody that exclusively marks the endothelium of small lymphatic vessels – E.Fulcheri, 2006). The cavernous lymphangiomas, as well as the skin and the subcutaneous forms, can affect the buccal mucosa, lips, and tongue with findings of macrocheilia and macroglossia.

They are located more frequently in the cervical and axillary regions, and more rarely in the limbs, eyelids, groin, and internal organs (lungs, intestines, retroperitoneum, etc.). They are formed by a network of large spaces covered by endothelium, empty or containing lymph, with a thick connective wall that may include muscular elements. Cystic lymphangiomas (or cystic hygromas) are almost always found in the neck and axillary regions. The mesenteric and retroperitoneal localizations are much more rare. They can reach considerable dimensions (up to over 15 cm in diameter). The tumor has a spongy appearance when it is constituted by a multilocular structure, but it can also present itself in the form of a single large cyst. Histologically, cysts are lined with flattened endothelial cells and separated by a thin fibrous web. Mesenteric lymphangiomas have ‘milky’ content (lymphangiomas chylosis). A particular variety of lymphangioma is the lymphangioma circumscriptum with cutaneous localization, of variable size, with possible involvement of the subcutaneous tissue, characterized by numerous and dilated lymphatic vessels in dermal papillae below the epidermis that shows a variable degree of acanthosis and hyperkeratosis. These lesions may appear on normal skin or on lymphedematous limbs and have a verrucous appearance, with possible ulceration and lymphorrhea.

The lymphangiosarcoma, macroscopically, appears in the form of multiple nodular efflorescences, with a nummular appearance and a dark blue-violet color, sometimes arranged in a ‘geographical map’ confluence and, often, filled with pus and hemorrhagic content. From the histopathological point of view, vascular structures are found with fusiform endothelial cells in cordoniform and partly reticular aggregates, often including lacunar zones, lined by mono- or multi-layered endothelium, with pericytes and fibroblasts. In areas where cells appear more compact, the presence of polymorphic nuclei with atypic mitosis is detected and in some areas the sarcomatous stroma is replaced by lymphatic sinuses and blood lacunae.

With regards to lymphomas, an essential feature of the morphological picture of Hodgkin’s disease is the subversion of the normal lymph node structure by a granulomatous-like process, in which a neoplastic cell component (H-RS cells) and a reactive one (lymphocytes, plasma cells, granulocytes, macrophages, fibroblasts) are recognized intimately committed. HD can be classified into 5 types: predominantly lymphocytic; nodular sclerosis; mixed cellularity; lymphocytic depletion; and a classic form rich in lymphocytes. For non-Hodgkin’s lymphoma, an histopathological classification is difficult to achieve due to the plurality of the histocytological pictures, the multiform clinical expressions, the diversity of the course and prognosis. The REAL (Revised European-American Classification of Lymphomas) classification represents an attempt to critically interpret lymphoma presentations based on all the information we have today on the lymphoma process: B-cell LNH, lymphoma deriving from B-cellular precursors (leukemia / B-lymphoblastic lymphoma) and lymphoma derived from peripheral B cells (B-cell chronic lymphatic leukemia, B-prolactocytic leukemia, small-cell lymphocytic lymphoma, lymphoma-lymphoplasmocytoma, or immunocytoma, mantle cell lymphoma, follicular center lymphoma, marginal zone B-cell lymphoma, monocytoid B-cell lymphoma, MALT-lymphomas, tricoleukemia, plasmacytoma, B-cell lymphoma, Burkitt’s lymphoma); LNH of T- and NK- cells, lymphoma deriving from T cell precursors (leukemia / T-lymphoblastic lymphoma) and lymphoma derived from peripheral T-cells (T-cell peripheral cells not otherwise specified, T-cell chronic lymphocytic leukemia, prolytic T-cell leukemia, granulated large lymphocyte leukemia), fungoid mycosis and Sézary syndrome, lymphoma-hepatic splenic T gamma-delta cell, subcutaneous T-cell lymphoma, angioimmunoblastic lymphoma, angiocentric lymphoma., primitive intestinal T-cell, associated with enteropathy, lymphoma / leukemia of the adult T-cell, l-large anaplastic cells).

Of particular surgical interest are MALT-lymphomas. These are neoplasms derived from mucosal lymphoid tissue (MALT), which includes Gut-Associated Lymphoid Tissue (GALT) and the Bronchus-Associated Lymphoid Tissue (BALT), both indicating organs in which mucosa physiologically present lymphoid structures. Subsequently, it was noted that in the stomach (although equipped with mucosa) and in some organs without mucosa, a MALT type tissue is absent under normal conditions but may be formed as a result of inflammatory-reactive or autoimmune events. Therefore, a primary MALT and a secondary MALT were distinguished (for example: secondary to H.pylori chronic follicular gastritis, Hashimoto’s thyroiditis, etc.). MALT may have uni- or multi-centric origin in the same bowel and a basic histopathological finding of low-grade MALT-LNH is the presence of lymphoepithelial lesions with center-like cells (CCL) cells, which invade and disintegrate the glandular-epithelial structures of the lymphoma site. Low-grade MALT-LNHs may evolve into high-grade forms of malignancy characterized by centroblastic and / or plasmoblastic and / or anaplastic morphology. 30-50% of the primitive extra-lymph nodal lymphomas arise in the gastrointestinal tract. They are almost exclusively LNH. Staging of primary lymphomas of the gastrointestinal tract includes: Stage I – Exclusive gastrointestinal involvement, without involvement of serosa (single primary localization or multiple, non-contiguous localizations); Stage II – Extension of the lymphoma from the primary gastrointestinal site to the lymph nodes (regional tributaries – II1 – or disseminated abdominals – II2); Stage III – outside of the serosa with infiltration of adjacent organs and / or structures; Stage IV – Disseminated extra-lymph nodal involvement or supra-diaphragmatic lymph node involvement.

 

In secondary tumors, because the afferent lymphatic vessels flow into the marginal sinuses, it is here that metastases are located. Subsequently, they spread to the whole lymph node which, however, may still preserve the capsule. Then, spread from lymph node to lymph node and, by extracapsular invasion, the affected lymph nodes adhere to each other and the tumor passes into the surrounding tissues. From the secondary lymphatic tumor, tumor cell clones can arise that, through the efferent lymphatic pathway, colonize other lymph nodes and can invade the blood stream (in particular, through the thoracic duct in the left supraclavicular site).

 

PHYSIOPATHOLOGY AND CLINICAL PICTURE

Simple lymphangioma is poorly differentiated from lymphangiectasia for both clinical and histopathological traits. It comes in various sizes, of whitish color, of soft consistency, easily depressible. The most frequent site is the subcutaneous tissue of the face and neck. Simple lymphangiomas grow slowly and usually do not cause significant disturbances, except for aesthetic damage. The cavernous lymphangiomas, on physical examination, appear as bluish-reddish areas, interspersed with translucent areas and points of increased consistency, together with areas that can be reduced by palpation. The localization in the tongue (macroglossia) and in the lips (macrocheilia) is classic. In addition to visible deformity, cavernous lympangioma in the oral site can be an impediment to speech, swallowing, and breathing. The main complications are represented by cutaneous or mucosal ulceration with consequent haemorrhage and/or lymphorrhea and recurrent lymphangitis. Cystic lymphangioma is predominantly located in the neck and rarely extends to the mediastinum, the axillary region, and the tongue. In most cases the symptoms are scarse. However, a forced position of opisthotonum in the child can result in difficulty in feeding and breathing. Dysphagia and dyspnoea may occur in the extensions to the tongue, the oral floor, and / or the mediastinum. Severe respiratory failure is more common in rare hourglass cervico-mediastinal cystic lymphangiomas. A sudden and considerable increase in the volume of the lymphangioma may occur when respitaory involement or hemorrhage occurs in the context of the tumor. Infection is a rare but very formidable complication and rupture can lead to a chylothorax.

The clinical course and the individual stages of development of Stewart-Treves lymphangiosarcoma are uniform. The disease affects a lymphedematous limb, starting with the spontaneous appearance of painful bruises, bluish nodules, skin plaques, papules, and ulcerations. Course and histopathology of lymphangiosarcoma are very similar to those that were described for the first time by Kaposi in 1872, reaching the denomination of sarcoma idiopaticum multiplex haemorragicum in 1894. Ackermann and Murray (1963), have shown that, in reality, it is of two substantially different clinical pictures. The most important difference concerns the prognosis. In Kaposi’s sarcoma many years, sometimes even decades, can pass between the manifestation of the initial efflorescence and the massive one of the nodules, while in the lymphangiosarcoma, death usually occurs within two years. Kaposi’s sarcoma mainly affects men, while lymphangiosarcoma is absolutely predominant in women (in relation to post-mastectomy lymphedema). Furthermore, Kaposi’s sarcoma tends to be associated with AIDS, in this case with a more poor prognosis.

Hodgkin’s disease is characterized by a clinical picture of variable onset from case to case, with the possible presentation in different stages of diffusion of the disease, with different locations, and with the presence or absence of general symptoms: low-grade fever or elevated and irregular thermogenesis, night sweats (especially in the back of the neck), generalized or district pruritus, deep asthenia, and weight loss. Constant is the early appearance of superficial and / or deep lymphadenomegalies. Among the superficial localizations prevail the latero-cervical and the supraclavicular (especially in the left side of the neck). The superficial lymph nodes have variable dimensions, characterized by the rapid increase in volume, hard consistency and smooth surface, indolent (characteristic, but infrequent, is the marked pain after ingestion of alcohol), mobile and isolated at the beginning, tending later to form packages and to adhere to the surrounding tissues. Mediastinal localization may cause a mediastinal syndrome due to involvement of the vascular and nervous, aerial and digestive structures of the mediastinum. Abdominal lymph node localizations are often clinically silent or cause general digestive disorders. In more rare cases they cause a peripheral lymphatic-venous stasis of the lower limbs and external genitalia. With regards to non-Hodgkin’s lymphoma, the onset, always insidious in all its forms, is sometimes characterized by a progressive symmetrical swelling of superficial lymph nodes in supra- and sub-diaphragmatic sites, other times (10%) by apparently isolated splenomegaly, or by slow anemia or the appearance of skin nodules. The superficial lymph nodes have symmetrical arrangement and usually modest dimensions, mobile, not painful, smooth surfaces, and parenchymatous consistency, do not adhere to the skin nor form packages, at least until advanced stages. Large retroperitoneal and mesenteric abdominal lymph node masses can be found. The Waldeyer ring and the tonsils can be hypertrophic, causing swallowing and phonation disorders. The spleen is usually enlarged and can reach significant sizes. Hepatomegaly is less common and it is rare to treat compression on the biliary tract from the lymph node packets of the hepatic hilum.

With regard to the clinical evaluation of nodules of a possible malignant neoplastic nature (eg: thyroid, breast, skin, etc.), it is always necessary to palpate the lymphoglandular satellite stations (cervical region, supraclavicular, armpit, groin), to confirm the existence or absence of lymphadenomegalies that may be related to the presence of a regional lymph node metastatic localization of the tumor itself.

DIAGNOSIS

As far as lymphangiomas are concerned, the diagnostic suspicion is based on the clinical features described above and the precise diagnosis uses ultrasound, CT, and MRI, which are also able to provide indications on the extent of the disease itself and the relationships that the neoformation has with neighboring structures. Lymphoscintigraphic examination may highlight the possible correlation of lymphangioma with the main lymphatic drainage pathways of the affected area, in particular for limb localizations. Such information is of fundamental importance from the preventive diagnostic point of view on the possible appearance of secondary lymphedema after surgical removal of the neoplasm.

In lymphangiosarcoma, biopsy of the cutaneous nodules with the clinical features described above is very useful and confirmed by CT and MRI. Also in this case, lymphoscintigraphy is able to provide useful information on the impairment of both superficial and deep lymphatic drainage of the affected limb or limbs, considering that in most cases lymphangiosarcoma appears on already lymphedematous limbs.

The investigations recommended to ascertain the diagnosis and extension (staging) of lymphomas (MH and LNH) include: Clinical and laboratory examinations (medical history, physical examination, routine laboratory tests such as dosage of Ig, serum immunoelectrophoresis, etc., immunological monitoring); Diagnostic imaging (chest x-ray, abdominal and/or superficial organ ultrasound, such as lymph nodes, thyroid, etc., thoracic-abdominal-pelvic CT, MRI, and other Rx-type, PET, lymphography, etc.); Routine invasive examinations (surgical lymph node biopsy, osteomyelobiopsia and bone marrow needle aspiration, rachicentesis and cytological examination of the CSF); Optional invasive examinations (laparotomy or laparoscopy with splenectomy and multiple biopsies, paracentesis of pleural effusion, pericardium, abdominal and cytological examination, digestive endoscopy).

Finally, with regards to the search for secondary lymph node tumors, ultrasound and CT scans are particularly useful, which allow a precise staging of the primary tumor, not only in terms of local size and extension of the lesion (T), but also in terms of lymph node (N) and distant (M) metastasis.

 

THERAPY

In small asymptomatic cystic lymphangiomas, immediate surgery has no indication. It is better to defer the surgical act to a more adult age to facilitate surgeon. The urgent surgical indication is posed only by the suppurative complication, which requires the incision and drainage, and by the acute respiratory insufficiency that necessitates the tracheotomy and / or the positioning of an aspiration drain under strict aseptic conditions. Sclerosing therapy of the neck lymphangioma is not recommended due to the risk of vascular or nervous lesions. The only effective therapy is surgical exeresis, which is rarely radical because the cystic lymphangioma, especially if it is extended, often has no sharp borders and infiltrates the surrounding structures, from which the risk of damaging important nervous structures (VII , XI, XII pairs of cranial nerves) is significant. For this reason, recurrence is not rare and it is necessary to perform repeated surgical interventions more or less close in time.

In the case of lymphangiosarcoma, surgical exeresis of nodules is often of palliative value, but lasting healing after extensive exeresis of single nodules is also known. However, the early diagnosis of a lymphangiosarcoma is the most important thing in the preventive and therapeutic sense, as well as prognostic. In the case of single nodules, with a histologically still-benign appearance, ie non-invasive or infiltrating, it will be possible to put in place, after a wide surgical excision, suitable radiotherapeutic procedures and a careful follow-up. In the presence, instead, of clearly malignant histopathological pictures, with a degree of dissemination such that it is easy to foresee a fulminant development, the amputation with the disarticulation of the limb will, then, be indispensable, as chemotherapy, both loco-regional (perfusion) isolated limb hyperthermia) and systemic (immunotherapy with interleukin-2), does not provide encouraging results, with an average survival of just 18 months.

As far as lymphomas are concerned, except for a small totally refractory group, they are generally susceptible to more or less complete and lasting control, if subjected to adequate therapy. The therapeutic means we have are represented by: ionizing radiation, cytostatic antiblastic drugs, immunotherapy, substances capable of modulating the body’s responses to the tumor (corticosteroids, interferon, etc.), transplantation of bone marrow or peripheral stem cells, surgical approach, etc. Regarding the surgical approach, the role of surgery in the treatment of lymphomas is mainly concerned with two aspects: staging (laparosplenectomy, particularly in Hodgkin’s lymphoma) and organ surgery (removal of tumor forms, more frequent among L. Non-Hodgkins). The surgical staging includes splenectomy, liver biopsy, removal of apparently increased lymph nodes, and applying metal reference clips for subsequent radiotherapy in each location. Therefore, the surgical staging is indicated when irradiation is necessary, while it appears useless when the treatment consists exclusively of chemotherapy (H at stage I and II, LNH at stage I). Splenectomy is indicated at any stage when severe splenomegaly is present and is contraindicated to below 15 years of age (risk of fulminant post-splenectomy sepsis). Surgery plays a fundamental role in extranodal lymphoma (organ tumor). The most affected organ is the stomach, particularly in the form of B-cell LNH. Long-term survival is greater than in carcinoma after aggressive surgery. Oncological surgical radicality has been shown to be dependent on tumor size, penetration, and spread, but not on the histotype. Lymphoma in the early stages (I and II) has a survival similar to that of the early cancer (90-95% at 5 years). In the more advanced stages (III and IV) one can still consider a surgical exeresis for an onco-reductive and symptomatic purpose, having provided surprisingly good palliative results even in the long term, due to the lower aggressive characteristics of lymphoma compared to carcinoma. Another rarer extranodal indication for LNH surgery is that of the small intestine, particularly the ileum. Generally, the clinical picture manifests itself as an occlusive complication or in relation to the appearance of a painful and palpable tumefaction, accompanied by weight loss. The resection of the affected intestinal tract is performed along with the lymphadenectomy in correspondence with the relative mesenteric range. Radical surgery of a malignant epithelial tumor generally involves extensive local-regional lymph node exeresis, which certainly plays a healing role, in case the spread of the neoplasm has stopped at this level, without haematogenous metastases at a distance, and has, however, an important meaning for prognostic purposes, as it allows one precisely stage the disease and to establish, therefore, the correct integrated therapeutic strategy (chemotherapy, radiation, etc.).

In lymphangiomas, percutaneous sclerotherapy has currently an important therapeutic role, in particular in microcystic tissue forms and cystic hygromas, as it gives good clinical results, in a non-invasive way, with the almost complete regression of vesicles or lymphatic lacunae. Depending on the size of the lesion, the most suitable sclerosing medium is chosen: in the smaller caliber forms, polydocanol is preferably used, in larger ones with large lymphatic spaces it is useful to use ethanol or Ethibloc. The injection of the sclerosing agent must always be followed by adequate local-regional selective compression, especially in large-sized lacunar forms.

Complementary therapies and alternatives to surgery are to be considered, in particular, in the treatment of lymphomas, whose therapy is based mainly on a combination of radiation and chemotherapeutics, associated with multiple adjuvants: blood transfusions in full or RBC concentrates or plasma enriched with platelets, anabolics, vitamins (A, C, folate), antiemetics, antibiotics and antibacterial and antifungal drugs, haematopoietic growth factors (erythropoietin), transplantation of bone marrow stem cells or autologous peripherals or genes etc. The sensitivity of lymphomatous cells to radiation and cytostatics and the eradicating efficacy of antitumor drugs are related to many variables (biological characteristics of lymphoma cells, extent and volume of the morbid process, etc.) and also the tolerance of the organism to anticancer therapies is in turn related to the age of the patient and the degree of impairment due to the lymphoma or for any previous illnesses.

With regard to radical lymphadenectomy for oncological purposes, conducted in critical locations such as the axilla and the groin, in the treatment of tumors of epithelial origin (breast, vulva, penis, melanoma, etc.), this lymph node surgery is not free from both early complications (lymphorrhea, lymphangitis, dehiscence of the suture) and late (lymphedema). Regarding the breast, for example, the incidence of secondary lymphedema of the upper limb in women undergoing mastectomy or quadrantectomy with axillary lymphadenectomy is equal 20-25%, up to 35-40% with the association of radiotherapy. Lymphedema of the lower limbs secondary to the treatment of tumors of the gynecological and urological sphere appears in a percentage ranging from 5% to 30%. Here we can easily understand the importance of an alternative method to radical lymphadenectomy, which is represented by the sentinel lymph node technique. Sentinel lymph node refers to the first lymph node receiving lymphatic drainage of the area of ​​the primary tumor and which is, therefore, the site of initial metastatic tumor implantation. This method allows a rational surgical approach to the regional lymph node stations and reduces the invasiveness of the surgery itself, significantly decreasing the incidence of the above mentioned complications, but still allowing an accurate histological and immunohistochemical evaluation of the lymph nodes at greater risk of metastasis; providing adequate staging of the disease.

Figure A

Figure B

Figure C

Figure D

Fig.1, A-D: Case of cystic lymphangioma of the left latero-cervical region.

Figure A

Figure B

Fig.2, A-B: Kaposi sarcoma of the right lower limb unrelated to HIV infection.

Dr. Stefano Spinaci is a specialist in Surgery and has dedicated himself to the treatment of breast disease already during his university and specialized training, participating in hundreds of operations of Breast and Oncological Surgery and Lymphatic Venous Reconstructive Microsurgery.

Spinaci is speaker and learner at numerous Congresses and Courses at international level and co-author of several scientific publications with significant impact factor.

Dr. Stefano Spinaci currently holds the position of Breast Specialist Director at Villa Scassi Hospital and at Asl of Genoa.

Breast cancer represents the most frequent neoplasia in women with incidence peaks in relation to age. In 2016, there were about 50.000 new cases registered in Italy alone. Breast pathology is not limited to malignant tumors, in fact, there are numerous alternations of a purely benign nature, such as Fibroadenoma, Mastis and Papillomas.

It is considered that one woman out of 12 will contract one of the aforementioned diseases over the course of her life.

Although the data may appear to be very relevant, it is essential to know that today an increasingly early diagnosis is possible thanks to the increasingly sophisticated technologies development, associated with the introduction of screening programs and highly specialized medical training courses, allowing surgical and therapeutic conservative treatments, minimally invasive and effective that guarantee the physical and psychological integrity of affected patients.

www.stefanospinaci.com 

 

Dr. Stefano Spinaci

Specialist in Surgery