Faculty Bibliography

Extranodal spread of lymphoma often affects the genitourinary system, with the kidneys being the most commonly involved organs. Contrast material-enhanced computed tomography (CT) remains the modality of choice for the detection, diagnosis, staging, and monitoring of renal lymphoma. Magnetic resonance (MR) imaging is particularly useful in patients in whom intravenous administration of iodinated contrast material is contraindicated. Ultrasonography (US), although very valuable for diagnosing lymphoma in the testis or epididymis, is less sensitive than CT and MR imaging for detecting renal lymphoma. Typical imaging findings of renal lymphoma include multiple poorly enhancing or hypoechoic masses, retroperitoneal tumors directly invading the kidneys, bilateral renal enlargement, and perirenal soft-tissue masses. Cystic lesions and tumors predominantly affecting the renal sinus and collecting system are uncommon. Unless the renal lesions manifest in the setting of widespread lymphoma, percutaneous biopsy is indicated to differentiate lymphoma from metastases, hypovascular renal cell carcinoma, uroepithelial carcinoma, or atypical infection, with US routinely being used to guide the procedure. Current immunohistochemical techniques allow accurate diagnosis and characterization of renal lymphoma. Radiologists should be familiar with both typical and atypical manifestations of renal lymphoma and should recommend imaging-guided percutaneous biopsy for diagnostic confirmation to avoid unnecessary nephrectomy.

Paraspinal masses (PSM) are uncommon and present a wide spectrum of differential diagnoses on fine-needle aspiration (FNA). We analyzed 59 cases of PSM on FNA in a 15-yr period, in the context of clinicoradiologic correlation. Radiologic findings, clinical data, and tissue biopsies were reviewed. Patients were 14-83 yr of age (mean 54.7) with a M:F ratio of 1.36:1. Of the 59 cases, 39 (66%) were deemed diagnostic. Of these, 8 (21%) revealed nonneoplastic lesions and 31 (79%) yielded neoplasms: 2 (6%) benign and 29 (94%) malignant. Of the malignant cases, 22 (76%) were metastatic tumors from various sites, while 7 (24%) were cancers from local spread, which included non-Hodgkin's lymphoma (NHL, 5) and myeloma (2). Benign neoplasms were nerve sheath tumors. Metastatic tumors consisted of adenocarcinoma, 9; squamous-cell carcinoma, 3; renal-cell carcinoma, 1; and non-small-cell carcinoma/not otherwise specified (NOS), 9. Twenty-four (41%) cases received further studies: immunoperoxidase (IPOX) alone, 17 (71%); special stains for microorganisms, 2 (8%); IPOX/other special stains, 4 (17%); and flow cytometry analysis, 1 (4%). Eight (14%) cases received follow-up biopsies. Half of these biopsies added information to previously "nondiagnostic" FNAs. Of the previously "diagnostic" FNAs, tissue biopsy yielded no additional information. Cytopathologic diagnoses were consistent with the pre-FNA radiology analyses in 13 (39%) cases. In instances of radiologic and cytopathologic discrepancy (4 cases, 12%), diagnoses made by FNA reversed the initial radiologic impression of neoplasm to infection, and vice versa. PSMs are rare lesions (0.26% of total FNAs done in 15 yr at our institution). The most common lesion encountered is metastatic adenocarcinoma, followed by NHL. Ancillary studies are helpful in difficult cases. In cases of radiologic/cytopathologic discrepancy, FNA diagnoses are more accurate and decisive for patient management. The sensitivity and specificity of a PSM FNA are 88% and 75% respectively.

The parapharyngeal space (PPS) is a well-defined anatomic zone of loose connective tissue lying deep to the tonsil and lateral to the pharynx. Neoplasms arising within the PPS are rare. We retrospectively reviewed 24 PPS fine-needle aspirations (FNAs) performed at The Johns Hopkins Hospital over the past 16 years (1987-2002). Patients presented with neck pain, dysphagia, and/or intraoral swelling of varying duration. Radiographic imaging disclosed PPS masses, varying in size from 2.5 to 8 cm. The most common clinicoradiographic suspicion was a nerve sheath tumor. Six cases had FNA performed using a 23-gauge needle via a transoral approach in the outpatient suite whereas the remainder were aspirated via a 22-gauge Franseen needle under CT guidance. Six of 24 cases (25%) were nondiagnostic due to lack of adequate cellular material. Of the 18 cases considered diagnostic, there were nine (50%) pleomorphic adenomas (PAs); three (17%) squamous-cell carcinomas (SCC); and one each of oncocytoma, adenocarcinoma, not otherwise specified (NOS), adenoid cystic carcinoma, lipoma, neurofibroma, and non-Hodgkin lymphoma, together comprising the remaining 33%. Four of the six cases deemed nondiagnostic (consisting predominantly of blood) on subsequent tissue follow-up revealed paraganglioma (two cases), SCC (one case), and schwannoma (one case). PPS is an uncommon target of an FNA procedure. PPS masses represent a heterogeneous group of neoplasms of which PA appears most common, representing 50% of our diagnostic cases. The rate of nondiagnostic FNA samples is moderately high due to excessive bleeding encountered in this location and other technical problems relating to adequately targeting the lesion in close vicinity of major neck vessels.

Recurrent respiratory papillomatosis (RRP) primarily is a disease of children and adolescents. Most patients experience spontaneous regression at a later age, while others continue to be affected throughout their lives. Here, we present the case of a 26-yr-old man who presented with persistent cough, worsening dyspnea, and development of pneumothorax. Eight years prior, he had presented with similar complaints and an evaluation at that time had showed RRP. Follow-up since that time showed recurrent disease managed by multiple surgical procedures. At the most current admission, an ultrasound-guided fine-needle aspiration (FNA) of the lung showed numerous papillary tissue fragments and single atypical squamous cells consistent with recurrent RPR. To our knowledge, morphological findings of RRP have been reported rarely.

BACKGROUND: Primary pancreatic lymphomas (PPL) are extremely rare. Clinically, PPL usually present with symptoms of carcinoma of the pancreatic head. An accurate cytopathologic diagnosis by fine-needle aspiration (FNA) is imperative because the primary treatment is nonsurgical, based on a combination of chemotherapy and radiation therapy. METHODS: Eight cases of PPL were identified from the pathology files of The Johns Hopkins Hospital over a 14-year period (1989-2003). All cases were diagnosed on FNA performed under radiologic guidance. Needle rinses were used to perform flow cytometric (FC) analysis. No tissue studies were performed after the FNA diagnosis was made. RESULTS: A strong male predominance (male-to-female ratio of 7:1) was noted. The patients ranged in age from 35-75 years (mean age, 55 years). The tumors varied in size from 2-14 cm, as evaluated on the radiologic scans (mean dimension of 8.0 cm). Abdominal pain was the most common presenting symptom (six patients) followed by jaundice, acute pancreatitis, small bowel obstruction, and diarrhea. The cytomorphologic features included hypercellularity with discohesive cells with round nuclei, often prominent nucleoli, mitoses, and karyorrhexis. By FC analysis, all eight cases demonstrated a monoclonal pattern of immunoglobulin light chain expression. The patients were treated with either chemotherapy alone or in conjunction with radiation therapy or stem cell transplantation. CONCLUSIONS: PPL is an extremely rare pathologic entity. FNA coupled with FC analysis appears to be highly accurate in the diagnosis of PPL and is the sole diagnostic modality used clinically. Based on cytomorphology, the main differential diagnoses of PPL involve secondary lymphoma, pancreatic endocrine neoplasm, and florid chronic pancreatitis. An accurate FNA diagnosis of PPL is critical for timely, nonsurgical management and obviates the need for an exploratory laparotomy.

Inflammatory pseudotumor (IPT) is a rare space-occupying lesion of unknown etiology that can mimic malignancy on clinicoradiological and pathological examination. A review of the cytopathology archives at The Johns Hopkins Hospital identified 12 cases from eight patients with histologically proven IPT (lung, seven patients; liver, five patients). There were six men and two women with an age range of 28-84 yr (mean age, 59 yr). Presenting complaints of IPT of the lung included shortness of breath and hemoptysis, and in cases of IPT of the liver complaints included abdominal pain and elevated liver function tests (LFTs). All cases were found to have mass lesions suspicious for a neoplasm on radiographic examination. Cytological specimens consisted of fine-needle aspiration (FNA; seven specimens) and bronchial brush/wash (five specimens). Diagnostic accuracy of cytology for IPT was low (5/12, 42%). IPT showed hypercellular smears (on FNA) with an admixture of various cell types including inflammatory cells with predominance of plasma cells, fibroblastic proliferation, granulation tissue formation, and atypical-appearing histiocytes with enlarged nuclei and intranuclear inclusions. Fibroblastic proliferation with mitoses may mimic mesenchymal neoplasms. Cytomorphology is nonspecific and IPT usually is a diagnosis of exclusion.

BACKGROUND: Hepatocellular carcinoma (HCC) is routinely graded histopathologically using a modified Edmondson system (ES). The cytologic grading of HCC has been used predominantly as an adjunct in differential diagnoses (i.e., to help distinguish HCC from other tumors as well as nonneoplastic lesions). However, there are unanswered questions regarding the reliability of the cytologic grading of HCC and its correlation with histologic follow-up. METHODS: A total of 106 cases of HCC were identified in the authors' cytopathology files from 1977 to the present. Of these cases, 64 had either a core needle or excisional biopsy sample that was judged to be adequate for histologic grading. From each case smears were graded independently in a blinded fashion by two cytopathologists, and tissue slides were graded by a liver pathologist. The cytopathologists' grading was then adjudicated by considering the histologic diagnosis as the "truth standard". Finally, after the scores were calculated, a statistical analysis was performed to ascertain the accuracy of the cytopathologic grading. RESULTS: The sensitivity for accurate grading was found to be highest for well differentiated (WD) lesions; the specificity was found to be highest for poorly differentiated (PD) HCC for both cytopathologists. Interobserver agreement was highest for WD HCC. WD HCC displayed cohesive fragments, often associated with characteristic vascular/endothelial patterns. In addition, moderately differentiated (MD) HCC demonstrated numerous single cells and atypical naked nuclei, usually with prominent nucleoli. PD HCC displayed loose nests and three-dimensional fragments (often gland-like), pleomorphism, macronucleoli, and focal necrosis. CONCLUSIONS: In the authors' experience, the three-tier cytologic grading of HCC was found to be only moderately accurate. The accuracy of cytologic grading was reported to be high for WD/PD HCC and low for MD HCC. The architectural criteria appear to be more useful for WD HCC, whereas marked cellular pleomorphism is specific for PD HCC. The authors propose that a two-tier grading system may be more useful, given the recent studies of HCC recurrence.