Haematologica 2002; 87:(06)ECR20
[Medline] [prev] [index] [next]Primary splenic lymphoma: diagnostic and therapeutic value of splenectomy
Luciano Izzo, Barbara Binda, Arianna Boschetto, Luciano Caramanico, Gaspare Galati, Enrico Fiori, Mario Marini,* Luigi Lo Mele,* Alessandro Stasolla*
Department of General Surgery "Pietro Valdoni" "La Sapienza" *Dept.of Radiology; University of Rome, Italy
Correspondence: Dr. Luciano Izzo, V. G.T. da Lampedusa, 9 00142 Rome Italy, Tel:06-49970215/333-1100866, Fax:06-49970215; E-mail: bia.leo@tiscalinet.it
Background: Primary splenic lymphoma is a rare disease. It can be defined as a lymphomatous process involving the spleen alone or the spleen and splenic lymph nodes .
Materials and methods: The authors report the case of a 75-year-old man who had complained for a few months ofasthenia and pain in the left hypochondrium and flank; the physical examination revealed massive splenomegaly and a swelling, of about 10-12 cm at the level of the left hypochondrium. Ultrasound and CT examination confirmed splenomegaly and a round splenic neoformation. The examination also showed the absence of evident pathological abdominal lymphadenopathies or focal lesions of other abdominal organs. The patient underwent total splenectomy and loco-regional lymphadenectomy. The histological exam confirmed the diagnosis of a non-Hodgkin's lymphoma without lymph node involvement.
Results: After 6 months the patient did not show any evidence of neoplastic loco-regional or distant recurrences.
Discussion and conclusions: Since the prognosis of these patients is linked to the degree of neoplastic diffusion (average survival after splenectomy: 82 months for primary spleen lymphomas versus 24 months for lymphomas extended to the liver or lymph nodal region beyond the splenic hilum), diagnostic imaging techniques, such as echography and CT, are indispensable for the depiction of splenic lesions and the possible involvement of other organs. Radical splenectomy and histological examination play both a diagnostic and a therapeutic role. A six-month disease-free period after surgery confirms the diagnosis.
Primary splenic lymphoma (PSL) is a rare pathology occurring in only 1-2% of cases1 in whom lymphomatous involvement of the spleen is present. According to Ahmann's criteria,2 we can identify three different types of involvement:
A lymphomatous process involving the spleen alone or the spleen and splenic lymph nodes (type 1 and 2, according to Ahmann) can be defined as a PSL.5 Histologically, it can assume four different macroscopic aspects:6
These lymphomas are usually of non-Hodgkin's type, originating from B-cells.5,7 Radical splenectomy together with related lymphadenectomy of the splenic hilum is the treatment of choice and a fundamental diagnostic tool. A six-month disease-free period after surgery confirms the diagnosis.8
We report here the case of a 75-year old man with PSL. The patient had complained for a few months of asthenia and pain in the left hypochondrium and flank. An abdominal ultrasound had shown splenomegaly and a round, hypoechogenic splenic neo-formation, with hyperechogenic areas within it, measuring 80x72x68 mm. Because of the worsening of his painful symptomatology and the onset of a painful swelling measuring approximately 7-8 cm in diameter, localized in the left hypo-chondrium, the patient was admitted to our Department. At hospitalization, he reported severe asthenia, a continuous low fever (37.2-37.5°C) for at least two months, and continuous pain in the left hypochondrium. The physical examination revealed massive splenomegaly and swelling in the left hypochondrium of about 10-12 cm in diameter, with a parenchymatous consistency and ill-defined margins. Blood tests were negative for HIV, HBV, HCV, EBV, or Cytomegalovirus infections; red cells were 4,360,000, Hb was 12.2, leukocytes were 11,900, platelets were 110,000 and total proteins were 6.51 g/dL. An abdominal ultrasound revealed the increased dimensions of the splenic neo-formation (maximum diameter: 12 cm), confirmed by a CT-scan of the abdomen and the pelvis: "remarkable splenomegaly: presence of a parenchymatous mass (maximum diameter of 12-13 cm) within the spleen, with polycyclic margins, hypodense, and moderately non-homogeneous. Absence of evident pathological abdominal lymphadenopathies and focal lesions of other abdominal parenchymatous organs." The bone marrow biopsy did not show lymph nodal infiltrations. The degree of splenomegaly and the risk of bleeding were considered contraindications to a percutaneous biopsy. The patient therefore underwent total splenectomy and loco-regional lymphadenectomy. The surgical exploration of the abdominal cavity did not show the presence of neoplastic spread. The histological exam of the spleen (22x18x8 cm) confirmed the diagnosis of a diffuse large B-cell non-Hodgkin's lymphoma (Figure 1); hilar lymph nodes were not involved in the neoplastic infiltration. After six months, the patient was in good general health without evidence of neoplastic loco-regional or distant recurrences. A definite diagnosis of PSL was therefore made.
PSL is difficult to identify because of its poor clinical symptomatology and rapid spread. Splenomegaly, pain in the upper abdominal quadrants, asthenia, anorexia, continuous low-grade fever, and night sweats are the hallmarks of the disease onset.5 Complete blood count (CBC) and peripheral blood smear are usually within normal limits, although cases of pancytopenia or leukocytosis can been recorded. Diagnostic imaging techniques are indispensable tools for the depiction of splenic lesions and possible involvement of lymph nodes or lymphomatous organs: ultrasound is usually able to show increased splenic volume and single or multiple splenic hypo-echogenic or non-homogeneous lesions, while the same lesions at CT imaging are hypo-dense sometimes with a necrotic center or hyperdense septa. These are, however, non-specific findings and represent a problem in differential diagnosis (metastases, hematomas, abscesses, angiosarcomas, hamartomas).6,9-11 The possibility of performing an US- or a CT-guided percutaneous needle biopsy12 should be carefully evaluated. Recent retrospective studies,13 in fact, report a major complication rate of 1% and a diagnostic accuracy of 90% (when performed by expert operators) considering cytological and microhistological sampling techniques. In our case, many reasons contraindicated a percutaneous biopsy. The extent of splenomegaly, the dimensions of the splenic mass, and the patient's symptoms have induced both surgeons and hematologists to perform a total splenectomy. This decision was also due to the fact that large and randomized studies on the advantages of chemo-therapy versus surgery in the treatment of PSL do not exist. Once splenectomy was decided, we did not believe it necessary to proceed with an invasive investigation. A bone marrow biopsy is considered a complementary diagnostic step in the pre-operative work-up. Total splenectomy and lymphadenectomy of the hilum, with exploration of the abdominal cavity, play both diagnostic and therapeutic roles. A definite diagnosis of PSL is made on the basis of the histologic examination of the whole organ and hilar lymph nodes. Radical splenectomy and eradication of the neoplastic mass determine the remission of the patient's symptomatology, in 82% of cases this corrects a cytopenia which characterizes the CBC in 77% of patients at the moment of diagnosis,14 and above all, in case of PSL, ensures long-term remission of the disease.5 The median survival rate after splenectomy in those subjects with type-1 or type-2 pathology, according to Ahmann's criteria, is approximately 82 months, regardless of the histologic type of the diagnosed lymphoma; in those patients with the type-3 disease, the median survival decreases significantly (24 months).5 A correct staging of the disease is therefore of vital importance together with the need to definethe diagnosis only six months after the surgical intervention, if recurrences do not occur during this period. Presently, internationally recognized agreement on the need to administer post-splenectomy adjuvant therapy does not exist.5,7,14 In our case, because of the age of the patient (75 years) and the radicalness of surgical ablation, chemotherapy was not prescribed. Eighteen months after surgery, the patient does not show any sign of tumor recurrence.
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