Fine needle aspiration cytology of primary lymphoid lesions of the orbit: report of four cases

BACKGROUND: The role offine needle aspiration cytology (FNAC) in diagnosis and differentiation of primary orbital lymphoid lesions is debatable, and little literature exists on cytodiagnosis of orbital lymphoid lesions. CASES: Four patients with orbital lesions underwent routine FNAC. In 3 cases, histopathologic examination was performed with immunohistochemistry (IHC). Cytologic features and diag- noses were correlated with histopathologic diagnoses. Two were cytologically diagnosed with low-grade non-Hodgkin lymphoma (NHL) and 2 with pseudolymphoma and inflammatory pseudotumor, respectively. Of the 2 cases of histologically diagnosed NHL, 1 had concordant diagnosis and the other had a jalse negative diagnosis of pseudolymphoma; both showed significant increase in mast cells, with neoplastie lymphoid cells exhibiting a higher N:C ratio and coarser chromatin texture. The case cytologically interpreted as suspicious for NHL was identified as a psetedolymphoma on histology (false positive). The case of inflammatory pseudotumor, in which hiopiy was not peiformned, responded well to corticosteroids, strengthening the cytologic diagnosis. CONCLUSION: FNAC diagnosis and differentiation of benign vs. malignant primacy orbital lymphoid lesions is often difficult. Careful cytologic interpretation with respect to certain subtle cytologic details is needed in minimizing false positive or false negative diagnoses. In difficult situations, IHC using suitable immunomarkers proves highly useful.     

Gastric Lymphosarcoma and Pseudolymphoma: Reappraisal of 12 Cases of Gastric Lymphosarcoma

Lymphoma localized to one organ is known to have a good prognosis. After a study of 131 cases of lymphosarcoma of the stomach Smith and Helwig³ reported that 42 were in reality a benign lymphoproliferative disorder which has been termed pseudolymphoma of the stomach. Of our series of 12 cases, six are now thought to be pseudolymphoma. Jacobs⁷ has enumerated the differentiating criteria. We have found a polymorphic cellular infiltrate, in nodular or diffuse pattern, associated with a fibrotic reaction located in the submucosa, muscularis or serosa, to be the most helpful criterion. The “good prognosis” associated with gastric lymphoma may be due partly to the difficulty in differentiating these two conditions and to the inclusion of cases of pseudolymphoma in any large series of these lymphomas.     

Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature

ABSTRACT: Angioimmunoblastic T-cell lymphoma (AITL) is one of the most common subtypes of peripheral T-cell lymphoma (15-20% of all cases), accounting for approximately 1-2% of all non-Hodgkin lymphomas. It often presents autoimmune phenomena including hemolytic anemia, thrombocytopenia, glomerulonephrities and circulating immune complexes (CIC). Polyarteritis nodosa (PAN) is an autoimmune disease characterized by necrotizing vasculitis of medium vessels, which rarely develops in association with hematological malignant disorders. Herein we report the case of a male patient with AITL who had a renal infarction secondary to PAN, mimicking a neoplastic lesion. A 40-year-old man underwent lymph node biopsy in the suspicious of sarcoidosis. On the basis of histological and immunohistochemical findings, a diagnosis of AITL was performed. The patient was successfully treated with a cytarabine-based regimen for 6 cycles. Three months after the initial diagnosis of AITL, a whole body CT-scan showed a lesion in the lower pole of the left kidney. A renal cell carcinoma was suspected, thus a nephrectomy was carried out. The histological findings were compatible with polyarteritis nodosa. To the best of our knowledge, the association between PAN and AITL has been described only once. This relation may be secondary to the induction of an autoimmune phenomenon by the lymphoma with the formation of circulating immune complexes, leading to vessel walls injury. A careful evaluation is needed in the management of AITL patients with signs of renal failure in order to avoid delay of treatment and organ damage. Key words: renal infarction, polyarteritis nodosa, T-cell lymphoma.     

Fine needle aspiration cytologic findings in pseudolymphoma cutis.

Skin nodules in three patients were sampled by fine needle aspiration. Cytologic study of the aspirated material showed a polymorphic cell population composed of small and large lymphocytes, eosinophils, plasma cells and tingible macrophages. Occasional giant cells and mast cells were also seen. These cytologic features suggested Hodgkin's lymphoma, lymphomatoid papulosis, large-cell non-Hodgkin's lymphoma and regressing atypical histiocytosis. However, because of the lack of typical Reed-Sternberg cells and due to the presence of polymorphic cells with fine chromatin, regular nuclear borders and inconspicuous nucleoli, these cases were diagnosed cytologically as a benign lymphoproliferative disorder, pseudolymphoma cutis. Biopsy of the lesions confirmed the cytologic diagnoses.     

Adult Wilms' tumor. Intraoperative cytology and ancillary studies performed in a case as an adjunct to the histologic diagnosis.

A case of blastema-predominant Wilms' tumor in a 64-year-old woman is reported. Intraoperative cytology of a renal mass was used to rule out malignant lymphoma and neuroendocrine carcinoma. Light and electron microscopy, immunocytochemical staining and flow cytometry (FCM) were also performed. Immunoperoxidase studies of smears showed positive staining for vimentin and negative staining for cytokeratins and epithelial membrane antigen. FCM DNA analysis of paraffin-embedded tissue showed no aneuploid peak. Frozen section interpretation of such tumors as seen in this case may be difficult, requiring distinction among several small-blue-cell neoplasms, including Wilms' tumor, neuroendocrine carcinoma and malignant lymphoma; intraoperative cytology can provide a valuable adjunct to frozen section diagnosis.     

Fine needle aspiration cytology of a low grade myxoid renal epithelial neoplasm: a case report

BACKGROUND: Recently, several case reports have described a rare but distinct subtype of renal tumor, referred to as a "low grade renal epithelial neoplasm," that appears to have a better prognosis than conventional renal cell carcinoma does. This report describes the cytologic features of this tumor as determined by fine needle aspiration (FNA) biopsy. CASE: A 53-year-old woman with a history of lymphoma had a renal mass incidentally discovered on an abdominal computed tomographic scan performed for lymphoma restaging. Results of an FNA biopsy showed relatively uniform, medium-sized tumor cells with moderate amounts of finely vacuolated or wispy cytoplasm and indistinct cell borders. The nuclei were primarily round with coarse chromatin and had prominent nucleoli. In the cell block preparation, the tumor cells showed a tubular architecture and an abundant myxoid matrix. The patient underwent a partial nephrectomy. The tumor was classified as a low grade myxoid renal epithelial tumor. CONCLUSION: This unusual kidney tumor appears to have distinctive cytomorphologic features, including a uniform population of epithelial cells with round nuclei, an abundant myxoid matrix and tubular architecture.     

Pituitary adenoma showing intermittent secretion of high molecular weight adrenocorticotropin without evidence of Cushing's disease

A 29-year-old woman was admitted in March 1998 due to high plasma ACTH levels, amenorrhea and uncontrolled diabetes mellitus (DM) which had persisted since 1991. Plasma ACTH levels showed a wide range of changes: they were usually high (59-240 pg/ml), intermittently very high (336-942 pg/ml), and sometimes normal or low. Plasma cortisol levels were usually normal but were sometimes high when the ACTH levels were very high. However, even when the plasma ACTH levels were very high, she did not show any cushingoid features. DM was diagnosed as non-insulin-dependent DM. Plasma ACTH showed an excessive response to CRH, while cortisol showed a delayed response. Plasma cortisol showed a poor response to ACTH-(1-24). ACTH receptor gene analysis revealed no mutations in the ACTH receptor-coding region. MRI showed a nonenhancing mass on the left side of the pituitary. Cavernous sinus sampling showed a very high plasma ACTH level in the left cavernous sinus compared with the levels in the right cavernous sinus and peripheral blood. Sephadex G-75 gel filtration of plasma ACTH immunoreactivity in plasma obtained by cavernous sinus sampling showed mainly high molecular forms of ACTH, probably proopiomelanocortin and ACTH-beta-lipotropin. This case is a very rare form of pituitary adenoma showing intermittent secretion of high molecular ACTH unaccompanied by cushingoid features.     

Sinus histiocytosis with massive lymphadenopathy: immunological, cytogenetic and molecular studies

We describe a case of "sinus histiocytosis with massive lymphadenopathy" (SHML) studied by immunohistochemical, cytogenetic and molecular analysis. The immunophenotyping showed that the lymph node histiocytes were strongly positive for the S-100 protein and MoAb LeuM3, OKM5, KP1 and DRC-1; a portion of these cells was also positive for OKT6 and Leu3A, suggesting a possible relationship with the veiled cells, which represent an intermediate step in the pathway from the Langerhans cell to the interdigitating reticulum cell. Cytogenetic analysis showed a normal prevalent clone and a small hypodiploid clone and the molecular study showed no detectable involvement of the c-fms proto-oncogene, which is related to monocyte/macrophages. Unfortunately all these data do not seem sufficient to define the benign or neoplastic nature of the disease. Further investigations, immunophenotypical, cytogenetic and molecular, are needed to elucidate the pathogenesis of the disease, especially for more aggressive cases or for cases with unfavorable evolution.     

Extranodal marginal zone lymphoma of the dura mater with IgH/MALT1 translocation and review of literature

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma involving brain, intraocular structures and spinal cord, without evidence of systemic disease. The majority of PCNSLs are diffuse large B-cell type. We encountered a rare case of primary dural marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) with extension into the brain in a 59-year-old man. A magnetic resonance imaging scan showed a 22-mm tumor located in the left posterior temporal lobe extending from the dura. Histopathology revealed a lymphoplasmacytic infiltration of the dura and the brain parenchyma in a perivascular pattern. Immunohistochemical and in situ hybridization studies showed a B-cell phenotype with kappa light chain restriction. Fluorescent in situ hybridization study showed a t(14;18)(q32;q21) with immunoglobulin heavy-chain/MALT1 fusion. The molecular study for immunoglobulin heavy-chain gene rearrangement by polymerase chain reaction showed a clonal gene rearrangement.     

Chronic spontaneous nephrocutaneous fistula associated with renal replacement lipomatosis

Chronic spontaneous nephrocutaneous fistula is a rare renal disease. Renal replacement lipomatosis (RRL) is the result of the atrophy and destruction of renal parenchyma with massive increases in the amount of fat in the sinus and perirenal space. The 2 conditions can be associated because they may have the same etiology. Indeed, urolithiasis is the most common cause of these diseases. We report a case of chronic nephrocutaneous fistula associated with RRL due to both urolithiasis and renal tuberculosis.     

Fine needle aspiration biopsy in the evaluation of lymphoreticular tumors of the thorax

The role of fine needle aspiration (FNA) biopsy in the evaluation of lymphoreticular disorders involving the lung and mediastinum was evaluated in a study of 16 cases from the authors' institutions. The cytologic material from these cases was reviewed, and the original cytologic interpretations were compared to the tissue diagnoses, with the overall accuracy of the cytologic interpretation evaluated. There were no false-positive diagnoses of malignancy in this series. A definite diagnosis of malignant lymphoma was most readily made in those cases in which the lymphoma was of the large-cell type. Small-cell and mixed large-cell and small-cell lymphoid proliferations were less able to be definitively interpreted, with pseudolymphoma presenting particular difficulty. The results support the conclusion that FNA biopsy can be useful in evaluating these lesions but also emphasize the fact that accurate diagnosis requires correlation of cytologic, clinical and laboratory data.     

Actinic reticuloid--photosensitivity or pseudolymphoma?--A review

Actinic reticuloid (AR) or chronic actinic dermatitis is considered a sunlight-induced pseudolymphoma (PSL) on light exposed areas of the skin, which primarily affects elderly males. The disease is a severe, chronic photosensitive dermatosis, first described by Ive et al. in 1969. PSL is a group of non-cancerous lymphocytic skin disorders that simulate malignant lymphomas, but the changes usually spontaneously regress. The clinical appearance of Actinic reticuloid is variable, usually characterized by an eczematous, pruritic eruption, predominantly present on the head and neck, or other sun exposed areas, but can involve any area of the body. Thereby, crucial characteristic is photosensitivity, where at action spectrum involves UVB, UVA and visible light beyond 400 nm. The disease is considered as PSL which histologically resembles lymphoma with immunohistochemical analysis of the cutaneous infiltrate revealing presence of activated T cells, numerous histiocytes, macrophages and B cells. Moreover, the development of malignant (non-cutaneous) T cell lymphoma in the course of AR has been reported. As the disease has chronic character, it requires significant changes in the patient's lifestyle and avoidance of provoking factors such as contact allergens or sources of intense light. Thus AR should be considered in every patient who presents with persistent, unclear, erythematous skin changes on the face and neck that are related to sun exposure.     

Human herpesvirus 8 in primary effusion lymphoma in an HIV-seronegative male. A case report

BACKGROUND: AIDS-related body cavity-based lymphoma, or primary effusion lymphoma (PEL), is a distinct clinicopathologic entity that occurs predominantly in immunosuppressed patients infected with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus. Although it rarely occurs in human immunodeficiency virus (HIV)-negative patients, we report such a case here. CASE: A 74-year-old male, who was HIV and Epstein-Barr virus (EBV) negative, was admitted to the hospital with dyspnea and chest pain. Chest radiography and computed tomography showed right pleural effusion. Cytologic analysis of the pleural effusion revealed a high grade lymphoma with round nuclei, prominent nucleoli and abundant cytoplasm. Polymerase chain reaction performed on the pleural effusion was positive for HHV-8 and negative for EBV. On molecular studies, the immunoglobulin heavy and kappa light chains were rearranged. Flow cytometry revealed a hyperploid fraction with DNA index of 1.29 expressing CD30. Immunostaining for HHV-8 from a cell block was positive. Electron microscopy revealed lymphomalike cells, many in various stages of apoptosis, with large nucleoli and clusters of viruslike particles in the nucleoplasm. CONCLUSION: A firm diagnosis of PEL can be established by the examination of cells from the lymphomatous effusion by a combination of cytology, molecular genetics, phenotypic features, immunostaining and electron microscopy. To our knowledge, this is the first case in which immunostaining for anti-HHV-8 monoclonal antibodies was used to support the diagnosis.     

The molecular weight of sodium hyaluronate in amniotic fluid

The molecular weight of sodium hyaluronate in human amniotic fluid was determined by gel chromatography. Pooled samples from 16 weeks of gestation exhibited a broad molecular weight distribution (Mw = 3 X 10(5); Mn = 6 X 10(4)). Samples taken at 40 weeks showed a low molecular weight fraction (M less than 10(5) presumably of fetal origin, and a high molecular weight fraction (M greater than 10(6) which varied considerably, indicating a nonfetal origin. No hyaluronate degrading activity was detected in the fluid. In a case of renal dysplasia, only minute amounts of hyaluronate were found. These findings are in accordance with a renal excretion of a low molecular weight hyaluronate from the fetus. In 12 cases with proven neural tube defects, there was no significant difference in hyaluronate level and molecular weight compared to normal controls.     

Cytologic diagnosis of peripheral T-cell lymphoma manifesting as ascites. A case report

BACKGROUND: Patients with malignant lymphoma seldom present with effusions without a known history of malignancy. Because of this, initial diagnosis of malignant lymphoma by effusion cytology is uncommon, with few reported cases. CASE: A 75-year-old male presented with fatigue, decreased appetite and progressively increasing abdominal girth over five weeks. Cytologic examination of ascitic fluid obtained by paracentesis revealed non-Hodgkin's lymphoma with a T-cell phenotype, confirmed by immunophenotypic and molecular studies. Approximately one week later, histologic examination of liver and bone marrow revealed involvement by lymphoma, demonstrating immunophenotypic and molecular profiles identical to those obtained from neoplastic lymphocytes recovered from the ascites fluid. CONCLUSION: This case demonstrates a rare presentation of peripheral T-cell lymphoma, clinically manifesting as ascites. In cases such as ours, where the effusion consists predominantly of small to intermediate-sized lymphocytes, distinguishing lymphoma from reactive lymphocytosis may be difficult. This case not only demonstrates the value of effusion cytology for lymphoma diagnosis but also illustrates how the use of various immunophenotypic and molecular techniques may assist the pathologist in properly diagnosing these difficult cases.     

Peripheral T-cell Lymphoma with Cyclin D1 overexpression: a case report

ABSTRACT: Peripheral T-cell lymphomas not otherwise specified are generally considered aggressive non-Hodgkin lymphomas, because of poor natural outcome and response to therapy. They show a complex karyotype without any specific genetic hallmark. We report a case of peripheral T-cell lymphoma not otherwise specified with heterogeneous nuclear Cyclin D1 immunohistochemical overexpression, due to gene copy gain, a phenomenon similar to that observed in Mantle Cell Lymphoma characterized by t(11;14)(q13;q32). In this case report we underline the diagnostic pitfall rapresented by Cyclin D1 immunoistochemical overexpression in a T-cell lymphoma. Several pitfalls could lead to misinterpretation of diagnosis, therefore, we underlined the need to integrate the classical histology and immunohistochemistry with molecular tests as clonality or Fluorescence in situ hybridization.     

Fatal primary diffuse large B‐cell lymphoma of the maxillary sinus initially treated as an infectious disease in an elderly patient: A clinicopathologic report

Objective

To report a case of primary diffuse large B‐cell lymphoma (DLBCL) of the maxillary sinus in an 82‐year‐old Caucasian woman.

Background

Diffuse large B‐cell lymphoma of the maxillary sinus has non‐specific signs and symptoms that may be confused with benign inflammatory conditions and upper respiratory infections.

Methods

An incisional biopsy was performed. CD20⁺/CD3^–/Ki‐67: 95% cells were observed.

Conclusion

A good medical history, clinical and imaging evaluations and immunohistochemical reactions are crucial to establish a correct and early diagnosis of DLBCL.     

A cytogenetic, phenotypic, and molecular study of an immunoblastic lymphoma with a 14q + translocation

An uncultured immunoblastic lymphoma, obtained from an untreated patient, was examined from a cytogenetic, immunophenotypic, and molecular viewpoint. The B-cell lineage, immunoglobulin light-chain type, and percentage of neoplastic cells were determined immunologically. Karyotyping showed the presence of a 14q + marker and suggested that the donor chromosome was chromosome 8. Southern-blot analysis of DNA from normal and lymphoma cells, using a molecular probes sequences related to the IGHJ and IGK immunoglobulin genes, confirmed the immunophenotype. A similar analysis, using probes homologous to IGHAC and MYC genes, showed that the t(8;14) detected by cytogenetic analysis resulted in a IGHAC-MYC rearrangement.