Localized reactive lymphoid hyperplasia of the spleen simulating malignant lymphoma. A report of seven cases

Since 1976, we have discovered isolated, solitary nodules in seven spleens due to reactive lymphoid hyperplasia. Six cases were encountered at staging laparotomy for malignant lymphoma and one case was observed in a spleen resected because of autoimmune hemolytic anemia. Macroscopically, the nodules were strongly suggestive of splenic involvement by lymphoma; microscopically, however, splenic lymphoma was not demonstrated in any case. In four spleens, the nodules were formed by focal aggregation of reactive germinal centers. In three other cases, the nodules were manifestations of a localized proliferation of lymphocytes, including immunoblasts and plasma cells. The immunoblasts raised the question of splenic involvement by Hodgkin's disease, but Reed-Sternberg cells were not identified. The etiology of localized splenic lymphoid hyperplasia is unknown, but the lesion is likely analogous to florid reactive follicular and diffuse hyperplasia observed in a solitary enlarged lymph node stimulating malignant lymphoma.     

Unique case of intramural colonic splenosis

Splenosis is usually a sequel of splenic rupture from abdominal trauma but can be associated with elective splenectomy. Recurrence of the hematological disorder for which the patient underwent splenectomy may occur, and splenic nodules can be found anywhere in the thoracic or abdominal cavity, as well as subcutaneously. We are presenting intramural colonic splenosis, a large inoculum of the splenic tissue that has been found to have the capacity to maintain anemia and thrombocytopenia, in a child previously splenectomized because of a hematological problem.     

Distinguishing splenosis from local recurrence of renal cell carcinoma using a technetium sulfur colloid scan

Heterotropic splenic tissue in renal fossa is characteristically asymptomatic and is usually an incidental finding that has been reported to mimic renal or adrenal tumors. A 55-year-old man with renal cell carcinoma had undergone radical nephrectomy together with splenectomy because of disrupture of the splenic capsule. During a follow-up examination, three nodules were detected by computed tomography scan in the splenorenal area and they slowly enlarged. Although local recurrence was highly suspected, we decided to rule out splenosis. We successfully diagnosed these masses as ectopic splenic tissues by a technetium sulfur colloid scan and unnecessary surgical exploration was avoided.     

Thoracic splenosis: Case report of a symptomatic case

Thoracic splenosis is the autotransplantation of splenic tissue in the left thoracic cavity as a result of a splenic injury. This rare pathology is usually asymptomatic and may be discovered on incidental imaging, but the diagnosis often requires invasive procedures such as surgery in order to eliminate a neoplasic origin. We report a rare symptomatic case of a 39-year-old man presenting with chest pain and multiple nodules revealed on a computed tomography scan. The patient underwent a surgical exploration and the pathological studies concluded to a thoracic splenosis. Indeed, the previous medical history of the patient revealed a left thoraco-abdominal traumatism during childhood. The aim of this paper is to emphasize that the diagnosis can now be performed using only imaging techniques such as technetium-99 sulfur colloid or labelled heat-denatured red blood cell scintigraphy to avoid unnecessary invasive procedures including thoracotomy.     

Complicated omental splenosis

Splenosis results from autotransplantation of splenic tissue, usually after traumatic splenic rupture. The diagnosis is suggested by the history and the presence of multiple nodules on computed tomography. Selective splenic scintigraphy utilising heat denatured red blood cells confirm the diagnosis. Treatment is usually conservative with surgical excision reserved for complicated cases. We report a case of splenosis associated with phlegmon and infectious process, requiring surgical intervention.     

Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion

Twenty-five cases of a morphologically distinctive vascular lesion of the spleen are described. The patients were 17 women and 8 men, ranging in age from 22 to 74 years (mean, 48.4 years; median, 56 years). The most common presentations were incidental finding of an asymptomatic splenic mass (13 patients), abdominal pain or discomfort (6 patients), and splenomegaly (4 patients). None of the patients had evidence of recurrent disease after splenectomy. The splenic lesion was solitary, measuring 3 to 17 cm, and sharply demarcated from the surrounding parenchyma. The cut surface revealed a mass of coalescing red-brown nodules embedded in a dense fibrous stroma. All cases showed a remarkably consistent multinodular appearance at low-power examination. The individual nodules had an angiomatoid appearance, in the sense that they were composed of slit-like, round or irregular-shaped vascular spaces lined by plump endothelial cells and interspersed by a population of spindly or ovoid cells. Some of the nodules (particularly the smaller ones) were surrounded by concentric rings of collagen fibers. Numerous red blood cells were present, as well as scattered inflammatory cells. Nuclear atypia was minimal, mitotic figures were extremely rare, and necrosis was consistently absent. The internodular stroma consisted of variably myxoid to dense fibrous tissue with scattered plump myofibroblasts, plasma cells, lymphocytes, and siderophages. Immunostaining revealed 3 distinct types of vessels in the angiomatoid nodules: CD34+/CD8-/CD31+ capillaries, CD34-/CD8+/CD31+ sinusoids, and CD34-/CD8-/CD31+ small veins, recapitulating the composition of the normal splenic red pulp. These features are therefore different from those of littoral cell angioma, conventional hemangioma, and hemangioendothelioma of the spleen. We interpret these angiomatoid nodules as altered red pulp tissue that had been entrapped by a nonneoplastic stromal proliferative process. The characteristic morphologic appearance, immunophenotype, and benign clinical course suggest that this is a distinctive nonneoplastic vascular lesion of the spleen that we propose to designate as sclerosing angiomatoid nodular transformation (SANT).     

Thoracic splenosis

Two rare cases of intrathoracic splenosis are reported. This phenomenon occurs almost exclusively in young men who have experienced splenic rupture and diaphragmatic laceration. The late occurrence of splenosis in the thoracic cavity poses problems in the differential diagnosis of solitary or multiple pleural-based nodules. It is important to recognize this entity from a clinical, radiographic, and therapeutic perspective.     

Influence of donor and host age on the regeneration and blood flow of splenic transplants

After splenectomy there is an increased risk of fatal overwhelming postsplenectomy sepsis, especially in children. If all alternatives to splenectomy fail, autotransplantation of splenic fragments is indicated. These fragments regenerate after a necrotic phase to small splenic nodules. Regulatory factors governing the regeneration process are largely unknown. Inbred rats were used as a model to define the influence of recipient and donor age on the regenerated mass and the blood flow of transplanted splenic fragments. These are both important factors for the protective function of the spleen. Fetal, newborn, weanling, or adult spleens were implanted into the greater omentum of newborn, weanling, or adult rats. The younger the recipient and donor, the better the regeneration and perfusion of transplants. However, these did not reach more than 40% of the normal splenic mass. In addition, no experimental group achieved more than one third of the normal splenic blood flow. There is an obvious age dependency in splenic regeneration and blood flow, but the transplants are far from attaining a normal splenic mass and perfusion.     

Splenic angiosarcoma: a case report with synchronous hepatic metastases

A 49 years old man in good physical condition suffering from abdominal pain in the right hypochondrium was hospitalized and CT scan revealed an hemoperitoneum, an enlargement of the liver and a rupture of an heterogeneous spleen. The patient was operated on and spleen removed. Pathological examination of the spleen concluded to a splenic cavernous hemangioma. In the postoperative course, an increase of liver nodules occurred within two weeks. A localized splenic angiosarcoma was recognized by reexamination of the specimen. The patient treated by chemotherapy was still alive after 5 months. Prognosis of splenic angiosarcoma is very poor; there is no curative treatment.     

Noninvasive methods of diagnosing thoracic splenosis.

Thoracic splenosis is a rare condition resulting from concomitant rupture of the spleen and left hemidiaphragm, with autotransplantation of splenic tissue into the left hemithorax. It is usually an incidental finding on chest plain film or computed tomogram and is rarely diagnosed without biopsy or operation. A history of old splenic trauma and findings of left-sided, pleural-based nodules should indicate the diagnosis, which can be confirmed with nuclear medicine studies.     

Splenosis after splenic rupture: a disease to be recognized]

One case of peritoneal splenosis is reported. The diagnosis was established at surgery in a 17-year-old female patient presenting with dull abdominopelvic pain, who had undergone total splenectomy after a trauma ten years earlier. This autograft of splenic tissue must be known to be acknowledged, especially today, when the conservative treatment advocated for ruptures of the spleen may increase its incidence. The literature reports only about one hundred cases. The treatment only consists in removing the sole symptomatic nodules.     

Littoral hemangioma of the spleen

The authors report a rare case of littoral hemangioma of the spleen (LHS) accompanied by a revision of the literature on the argument. A male 65-year-old patient was referred to their attention with suspected ultrasonographic diagnosis of lymphoma with a splenic localisation. The complete CT diagnosis led to suspected splenic angioma. During surgery, anatomopathological analysis of the biopsy revealed LHS. The pathological anatomy showed lesions ranging in size from small foci to large nodules which almost completely replaced the splenic parenchyma. These areas were made up of vascular canals or axes that imitate splenic sinuses and have irregular lumen, often appearing as papillary projections and cyst-like spaces; they are bordered by high (cylindrical) endothelial cells that project into the vascular lumen and reveal hemophagocytosis; there is very little mitotic activity. The patient was discharged 7 days after surgery. The authors underline the extreme rarity of this neoplasm and the virtual absence of symptoms, although some cases report signs of hypersplenism, including platelet deficiency and anemia. The diagnostic iter must take care to exclude other pathologies affecting the spleen, including lymphoma, metastases and primary malignant splenic tumours. Lastly, a differential diagnosis must be made with the malignant variant, littoral hemangiosarcoma of the spleen.     

Littoral cell angioma. A novel splenic vascular lesion demonstrating histiocytic differentiation

Seventeen cases of a novel type of vascular tumor of the spleen are described. The lesions, whose size ranges from minute foci to large nodules almost completely replacing the splenic tissue, are composed of anastomosing vascular channels resembling splenic sinus and have irregular lumina, often featuring papillary projections and cyst-like spaces; they are lined by tall endothelial cells that slough off into the vascular lumina and show hemophagocytosis. Atypical cells are absent and mitotic activity very low. In contrast to normal sinus endothelia, which express only FVIIIag, neoplastic cells express both endothelial (FVIII-AG, BMA 120) and histiocytic (KP1, lysozyme) antigens; occasionally S-100 protein is also present. The morphologic and immunohistochemical findings in this tumor reflect the dual differentiation potential of the reticuloendothelial cells lining the splenic sinus, justifying the term littoral cell angioma, and recognize a distinct entity that is different from other vascular lesions of the spleen, notably angiosarcoma. This distinction is all the more important because the clinical behaviour of this lesion is apparently benign.     

Splenosis mimicking local recurrence after radical nephrectomy: a report of two cases

Splenosis is autotransplantation of splenic tissue and usually follow traumatic or surgical rupture of the spleen. We report two cases of splenosis which presented as a local recurrence after radical nephrectomy for left renal cell carcinoma (RCC). The patients were a 65- and a 71-year-old male, who had been operated for RCC including splenectomy because of disrupture of the splenic capsule 8 and 9 years earlier respectively. In both cases, follow up computed tomographic scans showed small nodules under the left diaphragm. Although we initially suspected local recurrence, we considered the possibility of splenosis. Both patients underwent technetium-99m Sn colloid scans and were diagnosed with splenosis successfully. Therefore, we could avoid unnecessary surgical explorations.     

CT鉴别诊断脾原发血管性肿瘤的研究

目的评价CT检查对脾原发血管性肿瘤的临床诊断及鉴别诊断价值。 方法回顾性分析1989年12月至2018年11月经病理学证实的59例原发血管性肿瘤CT资料。 结果20例脾血管瘤增强扫描后可有3种强化方式;6例错构瘤表现与血管瘤类似;6例淋巴管瘤CT增强后无强化;17例血管淋巴管瘤依其淋巴管和血管构成比例不同而表现不一;8例窦岸细胞血管瘤表现为脾内多发低密度结节,呈渐进性强化或轻度强化;2例血管肉瘤表现为不均匀性强化,瘤内出现坏死和（或）异常肿瘤血管,常伴发转移。 结论脾原发血管性肿瘤影像学表现虽然各具特征,但部分征象相互重叠,CT检查有助于对其进行鉴别与诊断。     

Peritoneal splenosis. Report of a case and pathogenetic considerations

A clinical case of splenosis recently observed in a young man subjected at the age of 8 to splenectomy for rupture of the spleen and operated at 19 for intestinal occlusion is reported. The pathogenetic aspects of the condition, characterised by the presence of nodules of splenic tissue in the abdominal cavity, are examined through a review of the literature.     

A Rare Cause of Chronic Abdominal Pain: Recurrent Sub-torsions of an Accessory Spleen

Accessory spleen is defined as one, two, or three nodules of additional ectopic splenic parenchyma hung by a vascular pedicle generally near the spleen. Despite a relatively high frequency (from 10 to 30 % of the population based on autopsy studies), most accessory spleens are asymptomatic. Although cases of accessory spleen were clearly described in the literature, this perplexing diagnosis is often delayed and rarely made preoperatively. We repot episodic recurrences of abdominal pain in a 66-year-old man attributed to iterative sub-torsions of an accessory spleen, as well as a comprehensive review of the literature.     

肝硬化患者脾静脉和胃冠状静脉壁类粥样硬化样改变

研究肝硬化门静脉高压症患者脾静脉和胃冠状静脉壁类粥样硬化样改变,并探讨其发生机理及临床意义。方法５０例肝硬化门静脉高压症患者,在行脾切除贲门周围血管离断术时取一段脾静脉和胃冠状静脉供研究,１０例十二指肠球部溃疡患者和１０例外伤性脾破裂患者为对照组,行光镜和电镜观察。     

Micronodular T-cell/histiocyte-rich large B-cell lymphoma of the spleen: histology,immunophenotype, and differential diagnosis

Occasionally, primary large B-cell lymphomas (LBLs) arising in the spleen present with a micronodular pattern involving the splenic white pulp but sparing the red pulp. Histologically, the nodules contain scattered large B cells in a background of numerous T cells and histiocytes. They can cause substantial difficulty in histologic diagnosis as the morphology can mimic reactive and inflammatory lesions as well as other lymphoid neoplasms. In this study, we examined the histology and immunophenotype of the micronodular T-cell/histiocyte-rich LBL (MTLBL) of the spleen with a view to establish the characteristics that may be helpful in diagnosis. Paraffin-embedded material from 17 cases of MTLBL was studied. Clinical features and histology were reviewed and immunohistochemistry was performed for immunoglobulins, CD20, CD79a, CD3, CD68, CD10, BCL6, BCL2, OCT-2, epithelial membrane antigen, CD30, CD138, and EBV markers. The median age of presentation was 56 years, and the most frequent presenting features were anemia and B symptoms. All cases showed a micronodular pattern of involvement. The tumor nodules comprised a mixture of numerous CD3+ T cells and CD68+ histiocytes and scattered large CD20+ B cells with immunoglobulin light chain restriction. They were positive for BCL6 and OCT2 but negative for CD10, CD138, and EBV markers. There was variable expression of epithelial membrane antigen, Bcl-2, and CD30. No follicle dendritic cell meshwork infrastructure underlying the nodules could be demonstrated by staining for CD21 or CD35 antigens. The prognosis was poor; seven of the 12 cases with follow-up were dead within 2 years. MTLBL is unique variant of T-cell/histiocyte-rich diffuse LBL, characterized by primary splenic presentation and a micronodular architecture. The main differential diagnoses include granulomatous inflammation, Hodgkin's lymphoma, follicular lymphoma, and peripheral T-cell lymphomas.     

A rare diagnosis for a pancreatic mass: splenosis

Splenosis, the autotransplantation of splenic tissue, has been designed to preserve organ functions after splenectomy. We present the first case of laparoscopic resection of a pancreatic splenosis, in a patient who had undergone a splenectomy 31 years before, complaining of abdominal pain and diarrhea. Abdominal computed tomography (CT) scan showed an enhancing hypervascular 3-cm solid mass in the body of the pancreas, mimicking a pancreatic cancer or a neuroendocrine tumor. A diagnostic laparoscopy was planned, and a 3-cm peripancreatic nodule with a long pedicle was visualized, with many nodules close to the tail of the pancreas and in the greater omentum. They were all resected, and the specimens obtained were immediately sent for frozen-section examination, which confirmed the diagnosis of heterotopic splenic tissue. Splenosis should be included in the differential diagnosis of the pancreatic masses in patients with previous splenic surgery. A hypervascular mass on CT scan should be regarded as an adenocarcinoma of the pancreas until proven otherwise. The possibility of a neuroendocrine tumor mandates an octreotide scan and gastrointestinal hormones dosage. In the unlikely event that all tests may produce equivocal results, a diagnostic laparoscopy is mandatory, in order to obtain an accurate histopathologic diagnosis.