Thoracic splenosis diagnosed by fine-needle aspiration cytology: a case report.

A case of thoracic splenosis diagnosed by fine-needle aspiration (FNA) of subpleural lung lesions is presented. The patient, a 49-yr-old male with a history of gunshot wound to his trunk with splenic rupture and splenectomy several years previously, presented with recent hemoptysis and multiple subpleural solid nodules shown in the left lung field by CT scan. As the possibility of a metastatic malignancy vs. mesothelioma was entertained, an FNA was performed on one of these lesions, revealing lymphoid tissue with abundant vascularity simulating the structure of splenic tissue. In view of this finding, supported by the absence of splenic outline in the CT radiograph, the diagnosis of splenosis was made. This is the fourth reported case of thoracic splenosis in which FNA cytology was utilized for diagnosis, yet the first in which the diagnosis was based exclusively on the FNA cytologic findings. Clinical, pathologic, and diagnostic aspects of this entity are discussed.     

Unusual subcutaneous splenosis occurring in a gunshot wound scar: pathology and immunohistochemical identification

Splenosis usually occurs after traumatic rupture of the spleen with autotransplantation of splenic tissue to ectopic sites. Most commonly, it occurs as intraperitoneal nodules, which are found either incidentally or after symptomatic complications. Subcutaneous splenosis is a rare condition mostly observed in abdominal surgical scars. Reported herein is a case of subcutaneous splenosis developed in an exit gunshot wound scar on the left lower chest wall 9 years after splenectomy. The lesion presented as an asymptomatic subcutaneous nodule, which was excised under the impression of a skin tumor. Microscopically, there were multiple subcutaneous nodules resembling splenic tissue with red and white pulp. They were confirmed to be ectopic splenic tissue by immunohistochemical staining of the vascular lining cells (CD8+, CD31+, and CD34-). Only 11 cases of subcutaneous splenosis have been reported before. The present case was the third case occurring in an exit gunshot wound scar, and the diagnosis was confirmed by immunohistochemical study.     

Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour of intermediate behaviour. It can arise from various sites including the liver, spleen, pleura, or lung. Cutaneous EHE can be primary or secondary. This report describes the case of a 51 year old man who presented with a history of dry cough, shortness of breath, and pleural effusion, and who developed two cutaneous nodules in the anterior abdominal wall a few weeks later. He had a previous history of asbestos exposure. Computed tomography scan showed a left sided pleural effusion and nodular pleural mass. Histology of both the pleural and cutaneous lesions was compatible with EHE. Electron microscopic examination demonstrated the presence of Weibel-Palade bodies. The patient underwent elliptical excision of the metastatic cutaneous nodules after decortication of the primary pleural tumour and adjuvant treatment. A few reports have described metastasis of intrathoracic EHE to the skin. Despite treatment with interferon, the patient developed more cutaneous lesions two years after the initial diagnosis. Even though the tumour has the classic light histological and ultrastructural features of EHE, it behaved in an aggressive manner.     

Intrahepatic splenosis mimicking liver cancer: report of a case and review of literature

Intrahepatic splenosis is a rare situation occurred after traumatic splenic rupture or splenectomy. We report a 33 year old man with multiple isolated liver masses indistinguishable from primary and metastatic liver tumor by standard evaluation. The man underwent a splenectomy due to trauma at the age of three so that the hepatic splenosis was suspected. The final fine-needle biopsy pathological examination proved the diagnosis of intrahepatic splenosis. The importance of suspicion for this diagnosis should be highlighted when tumor-like lesions disclosed on imaging occurs in a patient with a splenic injury in the past. ^99mTc labelled heat-denatured erythrocyte scintigraphy can be helpful to the diagnosis since it may avoid the performance of biopsies or surgical resections.     

Splenic tissue in the ovary: Splenosis,accessory spleen or spleno-gonadal fusion?

Ectopic splenic tissue in any location could represent accessory spleen, a common congenital abnormality, splenosis, the presence of numerous nodules of ectopic splenic tissue after spleen trauma/surgery or splenogonadal fusion, a rare congenital malformation found mostly in males. Ectopic splenic tissue in the ovary is usually found in the context of an extensive pelvic splenosis. Solitary ovarian splenosis presenting not as a part of pelvic splenosis, but rather as a solitary mass masquerading an ovarian tumor is extremely rare. Differential diagnosis in this case includes mostly splenosis and splenogonadal fusion of the discontinuous type. Relevant literature and possible pathogenesis are discussed.     

Multiple calcifying fibrous tumors of the pleura

Calcifying fibrous tumor (CFT) is a rare lesion characterized histologically by hypocellular hyalinized collagenous tissue with psammomatous and/or dystrophic calcifications and patchy lymphoplasmacytic infiltrates. CFT usually occurs in the somatic soft tissue of children and young adults but is rarely found in the pleura. We describe here an unusual case of multiple small CFTs in the right mediastinal pleura of a 54-year-old man who had a history of renal cell carcinoma. Suspecting pulmonary and pleural metastases, we performed wedge resection of the right middle lobe and local excision of two nodules in the right pleura. Light microscopy revealed metastatic lesions of renal cell carcinoma in the resected wedge. The pleural nodules were well circumscribed and composed of hypocellular, dense, hyalinized, collagenous tissue with scant lymphoplasmacytic infiltration and characteristic psammoma bodies. Immunohistochemical staining revealed that most spindle cells were positive for vimentin, CD34 and factor XIIIa, and negative for epithelial membrane antigen, keratin, smooth-muscle actin, desmin, S-100 protein and anaplastic lymphoma kinase. We made a histological diagnosis of CFT of the pleura, and the patient remains well 6 months after the wedge resection.     

A unique case of bilateral ovarian splenosis and review of the literature

Splenosis is an acquired anomaly related to heterotopic auto‐transplantation of splenic tissue following abdominal trauma or splenectomy. We report the first definitive bilateral ovarian case in a 65‐year‐old woman who underwent splenectomy following a motor vehicle accident 44 years prior to presentation. We review the literature and discuss the main differential diagnoses. Gross examination revealed a 1‐cm well‐circumscribed dark nodule on the surface of each ovary. Paraffin‐embedded, formalin‐fixed blocks were sectioned and stained with hematoxylin–eosin and immunostains (CK5/6, Calretinin, WT1, Vimentin). The histological presence of both red and white splenic pulp, delimitation from ovarian tissue and ovarian origin of blood supply, as well as medical history, led us to the correct diagnosis. The outer nodular surface was covered by mesothelium (WT1+, CK5/6+, Calretinin+, Vimentin+), which was in continuity with the ovarian surface epithelium. To our knowledge, only six previous cases of ovarian splenosis are reported. Our patient is the oldest, with a very long interval from splenectomy to presentation. Clinically, splenosis may mimic malignancy, and a correct diagnosis avoids unnecessary overtreatment. The differential diagnosis includes an accessory spleen, spleno‐gonadal fusion, and splenic hamartoma: they should be excluded to come to the correct diagnosis.     

Multiple cavernous hemangiomas of the lung: a case report and review of the literature

Cavernous hemangiomas are benign vascular tumors most commonly seen in the head and neck region in childhood. They have been described rarely in the lungs. We describe a patient with incidental pulmonary nodules discovered at autopsy, which measured up to 0.9 cm and which were present in the lung parenchyma, as well as on the pleura. The nodules were composed of dilated vascular spaces lined by flattened bland cells. Immunohistochemical studies of the lining cells revealed CD34 and factor VIII immunoreactivity, consistent with a lesion of endothelial origin. Taken together, the gross, microscopic, and immunohistochemical findings support the diagnosis of multiple pulmonary cavernous hemangiomas.     

Pulmonary nodular elastosis: The intraparenchymal counterpart of pulmonary apical caps?

We have studied six cases in which focal consolidative pulmonary opacities observed on imaging studies led to surgical resection due to the suspicion of malignancy and showed on histopathologic examination a benign process characterized by an expansile tumor-like nodular accumulation of elastotic material. The patients were five women and one man aged 46 to 67 years (mean: 61 years). All lesions were found incidentally on imaging studies done for a variety of reasons, including surveillance for metastatic carcinoma in four patients. The lesions presented as solid nodules within lung parenchyma with irregular borders and spiculated margins and measured between 0.6 and 4.6 cm in diameter. Histological examination showed dense deposits of elastic tissue without evidence of malignancy, similar to those seen in pulmonary apical caps. Clinical follow-up between 5 and 16 years (mean: 10 years) showed that all patients were alive and well without evidence of disease. Pulmonary nodular elastosis is a localized intraparenchymatous process that may be confused clinically and radiographically for a malignant neoplasm and needs to be distinguished from other nodular lesions of the lung. To the best of our knowledge, tumor-forming lesions within lung parenchyma that are predominantly or almost exclusively composed of accumulation of elastic fibers have not been previously described.     

Primary pleural angiosarcoma associated with pneumoconiosis: An autopsy case

We report a case of pleural angiosarcoma in an adult male patient confirmed by autopsy and possibly associated with pneumoconiosis. The lesion was characterized by thickened pleura of both lungs with nodular tumors. Histologically, the tumor was composed of spindle‐to‐polygonal epithelioid cells that were positive for CD31, CD34, vimentin, and cytokeratin on immunohistochemical staining but were negative for calretinin. Further examination revealed mix‐dust pathological findings consistent with the existence of pneumoconiosis; dystrophic ossification, anthracosis, and fractal small dust particles were observed in the lung parenchyma and a hilar lymph node. The current case suggests that pneumoconiosis‐associated pathologies may be risk factors for the development of angiosarcoma in the pleura.     

Oncocytic nodular hyperplasia of the thyroid

Nodular hyperplasia of the thyroid is a process whereby the gland experiences growth by nodular expansion of thyroid parenchyma. We have encountered 45 patients in whom the process was caused by the growth of well-defined and sharply circumscribed but unencapsulated nodules composed of oncocytic thyroid follicular cells. The lesions arose in 39 women and 6 men, aged 25–69 years (mean = 50.3 years). The surrounding thyroid parenchyma showed features of chronic lymphocytic thyroiditis. The nodules varied from microscopic to 5 cm and appeared to compress the surrounding thyroid parenchyma. Most of the lesions lacked a well-defined capsule. In 26 tumors, the nodules displayed a predominantly follicular pattern of growth; in 8 cases there were admixtures of follicular and trabecular patterns with focal solid areas devoid of follicles. Clinical follow-up in 39 patients ranging from 7 to 22 years (median = 16 years) showed no evidence of recurrence, metastasis, or malignant transformation. One patient died of unknown causes 15 years after the diagnosis, and another patient died 4 years after diagnosis from metastatic colonic adenocarcinoma. Oncocytic nodular hyperplasia is a benign process associated with chronic lymphocytic thyroiditis that should be distinguished from benign and malignant oncocytic (Hurthle cell) tumors of the thyroid.     

Pleural epidermoid carcinoma from displaced skin following extrapleural pneumothorax in a patient exposed to asbestos

This report illustrates a rare case of primary epidermoid carcinoma of the pleura in a patient previously treated by artificial extrapleural pneumothorax for active tuberculosis. The patient had also been occupationally exposed to asbestos. Light microscopic examination showed two different lesions: laminar pleural fragments were covered by normal squamous epithelium that was similar in all respects to epidermis, whereas nodular fragments were composed of well-differentiated infiltrating carcinoma. These findings support the hypothesis that the carcinoma arose from normal epidermis seeded in the pleural cavity during multiple air refills to maintain the pneumothorax. A possible interaction between asbestos fibres and chronic inflammation might have potentiated tumour development.     

Pleuro-pulmonary endometriosis and pulmonary ectopic deciduosis: A clinicopathologic and immunohistochemical study of 10 cases with emphasis on diagnostic pitfalls

The clinical and pathological features of nine cases of pleuropulmonary endometriosis and the first case of pulmonary ectopic deciduosis are presented. The patients were all women between the ages of 27 and 74 years (median, 36 years) who presented with symptoms of catamenial pleural pain, shortness of breath, hemoptysis, or radiographically detected lung masses. Clinically, six patients were multiparous, one patient had pelvic endometriosis, and four patients had undergone prior pelvic surgical procedures, including dilatation and curettage. Radiographically, eight patients had pulmonary infiltrates or nodules, and four patients had pneumothorax. Three cases involved the visceral pleura and one case the parietal pleura. The other six cases, including the single case of ectopic deciduosis, involved the lung parenchyma. Histologically, the single or multifocal lesions were well circumscribed or infiltrative, nodular, cystic, or nodulo-cystic, and showed the characteristic features of proliferative or secretory endometrium with numerous müllerian metaplastic changes. Mucin stains were negative in five cases of endometriosis and in the single case of ectopic deciduosis. Immunohistochemical studies were performed in these same six cases using antibodies to epithelial, mesenchymal, vascular, and neuroendocrine markers. The glandular epithelium was decorated with antibodies to pan-cytokeratin, CK7, BER-EP4, ER, and PR, whereas the stromal cells showed positive staining for vimentin, actin, smooth muscle actin, desmin, ER, and PR. Follow-up information obtained in seven patients showed all women without recurrences after 1 to 20 years. The current study highlights the importance of recognizing intrathoracic endometriosis and ectopic deciduosis and properly assessing small biopsy specimens to avoid a misdiagnosis of malignancy.     

Pleural MALT lymphoma diagnosed on thoracoscopic resection under local anesthesia using an insulation-tipped diathermic knife

A 79-year-old man presented with back pain. Chest CT scan showed elevated nodular lesions in the right parietal pleurae with pleural effusion. There were no intrapulmonary or mediastinal abnormalities. Under local anesthesia, right thoracoscopy and subsequent thoracoscopic pleural resection were performed using an insulation-tipped diathermic knife (IT-knife). The resected pleura, 2.2 cm in diameter, had a rough granular surface. Lymphoid cells histologically infiltrated diffusely into the pleura. They were composed of centrocyte-like and monocytoid cells. On immunohistochemistry they were found to be positive for Bcl2, CD20, CD45RB and CD79a, but negative for CD3, CD5, CD10 and cyclin D1. EBV-encoded small RNA-1 (EBER-1) in situ hybridization was negative. A diagnosis of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) arising in the pleura was therefore made. To the authors' knowledge this is the first case in which IT-knife was used for diagnosis of a pleural lesion. This large, single-piece, only slightly crushed pleural specimen, enabled study of histopathological findings (listed here) that could not have been obtained on conventional biopsy: (i) lack of apparent evidence of plasmacytic differentiation; (ii) no recognition of lymphoid follicles; (iii) mesothelial cells not infiltrated by lymphoma cell clusters; (iv) thin layer of hyperplastic mesothelial cells continuously covering the surface; and (v) no proliferation of fibroblast-like submesothelial cells.     

Diffuse intrapulmonary malignant mesothelioma presenting with miliary pulmonary nodules: A case report

A 67‐year‐old male with a history of asbestos exposure presented with fever, cough, and dyspnea and was found to have diffuse granular shadowing in both lungs, right pleural effusion, and hilar and mediastinal lymphadenopathy upon chest computed tomography. For definitive diagnosis, a thoracoscopic lung biopsy was performed. Intraoperative findings showed no remarkable macroscopic changes in the visceral and parietal pleura, although a high level of hyaluronic acid in the pleural effusion was noted. Histological findings showed proliferation of atypical cells with round‐to‐oval nuclei, prominent nucleoli, and eosinophilic cytoplasms. These cells were arranged into sheets or tubules and were located predominantly in the lung parenchyma. Lymphovascular invasion was conspicuous. Immunohistochemically, tumor cells were positive for calretinin, D2‐40, and CK5/6, focally positive for Ber‐EP4, but negative for WT‐1, TTF‐1, CEA, and MOC31. Fluorescence in situ hybridization for the tumor suppressor p16 revealed homozygous deletion in the tumor cells. Therefore, we diagnosed the tumor as diffuse intrapulmonary malignant mesothelioma (DIMM). The patient had a poor response to chemotherapy and died 1 year after diagnosis. Although rare, DIMM should be considered when patients present with multiple, tiny intrapulmonary nodules, regardless of macroscopic pleural changes. Furthermore, this is the first report on p16 status in DIMM.     

Hepatic splenosis preoperatively diagnosed as hepatocellular carcinoma in a patient with chronic hepatitis B: a case report

We report on a case of hepatic splenosis. A 32-yr-old man underwent a splenectomy due to trauma at the age of 6. He had been diagnosed as being a chronic hepatitis B-virus carrier 16 yr prior to the surgery. The dynamic computer tomography (CT) performed due to elevated serum alpha-fetoprotein (128 ng/mL) demonstrated two hepatic nodules, which were located near the liver capsule. A nodule in Segment IVa had a slight enhancement during both the arterial and portal phases, and another nodule in Segment VI showed a slight enhancement only in the portal phases. Dynamic magnetic resonance imaging (MRI) of the mass in Segment VI showed enhanced development in the arterial phases and slight hyperintensivity to the liver parenchyma in the portal phases. These imaging findings suggested a hypervascular tumor in the liver, which could be either focal nodular hyperplasia, adenoma, or hepatocellular carcinoma (HCC). Even though these lesions were diagnosed as HCC, some of the findings were not compatible with typical HCC. On dynamic CT and MRI, all lesions showed a slight arterial enhancement and did not show early venous washout. All lesions were located near the liver capsule. These findings, along with a history of splenectomy, suggested a diagnosis of hepatic splenosis.     

Calcified pseudotumor of the pleura: description of a case

We describe a calcifying fibrous pseudotumour of pleura in a 46-year-old female, smoker. The patient presented with a well-delimited pleural mass, 3-cm across, located at the base of the right lung and attached to the lung with a short pedicle. Seven years after surgical excision of the mass, the patient is alive and well. Microscopically, the lesion was mostly composed of dense collagenous tissue, with sparse benign spindle cells, a rich inflammatory infiltrate and scattered calcifications, sometimes laminated. Immunohistochemically, spindle cells were positive for vimentin and negative for smooth muscle actin, desmin, S100 protein, CD34, CD99 and Bcl2. Calcifying fibrous pseudotumour is rare in the pleura. Pertinent data from the literature and problems in differential diagnosis are briefly discussed.     

Hepatic splenosis diagnosed by fine-needle cytology

A case of hepatic splenosis, diagnosed by FNA cytology, in a 56 years old cirrhotic man is reported. The cytologic material, aspirated from a 4.8 cm "suspicious for hepatocarcinoma" nodule, showed, within a hematic background, small-medium sized lymphoid cells, neutrophils and eosinophils granulocytes, histiocytes with large cytoplasm, plasma cells; mixtures of these cells, within a reticular network, were often aggregated in large structures, in which endothelial cells organized in vascular-like structures were also visible. The cell block, showed sinusoidal structures, lymphoid follicular aggregates and fibrous septa, strongly suggesting the micromorphology of the normal spleen. Splenosis is an autotransplant of splenic tissue, morphologically organized in non-capsulated, functioning, self-limited nodules, secondary to a post-traumatic splenectomy. Usual presentation of this condition is the presence of several bluish nodules on the peritoneal surface. Nevertheless, sporadic cases in other organs have been reported. To the best of our review of the Medical Literature, a hepatic splenosis is an exceptional even and the present is the first case in this location diagnosed by FNA cytology. The present one is also the case with the largest latency between the splenectomy and the splenosis detection (43 years).     

Isolated pleural PTLD after cardiac transplantation

Preamble: Epstein–Barr virus infection (EBV) and immunosuppression promote emergence of posttransplant lymphoproliferative disorders (PTLD) in patients undergoing organ transplantation. Objective: We report a case of PTLD confined to the pleura. Findings: The patient was a 62-year-old male who had undergone cardiac transplant in 1993 for ischemic heart disease. Seven years later, he presented with dyspnea and bilateral pleural effusions. The CT scan revealed left sided pleural base thickening. The cytology of the pleural fluid and fine needle aspirate of the pleura was both suggestive of PTLD. However, the tissue submitted for ancillary studies did not contain the diagnostic material. A clinical decision was made to withdraw immunosuppressive therapy and start rituximab. His clinical course was complicated by Pneumocystis carinii pneumonia and he died 4 months after the diagnosis of PTLD. Autopsy revealed bilateral pleural effusions with pleural nodules involving the visceral and parietal pleura of both lungs. Immunohistochemistry demonstrated B cell lineage with κ/λ ratio of 1. PCR studies done on the pleural nodules (postmortem specimen) revealed the presence of EBV DNA and absence of human herpes virus 8 (HHV8) DNA. In situ hybridization revealed positive staining for EBV RNA within the neoplasm. Conclusion: Pleural-based PTLD is rare. Cytology in conjunction with immunophenotyping and molecular studies can be useful for a definitive diagnosis. In our case, cytology sample was suggestive of PTLD. PCR studies performed on the antemortem specimen confirmed the presence of monoclonal IgH gene rearrangement, while the postmortem specimen revealed oligoclonal IgH gene rearrangement. The change from monoclonal to oligoclonal IgH gene rearrangement suggests reversion of monoclonal to polyclonal PTLD following rituximab and CHOP therapy. We also demonstrated EBV DNA and RNA in the tumor nodules, supporting EBV-induced PTLD.