Parathyroidectomy promotes wound healing and prolongs survival in patients with calciphylaxis from secondary hyperparathyroidism.

BACKGROUND: Calciphylaxis is a rare but life-threatening condition occasionally affecting patients with secondary hyperparathyroidism. Parathyroidectomy has been advocated as the only potentially curative intervention. METHODS: Between January 1989 and May 2000, 13 patients with pathologic/clinical criteria of calciphylaxis were treated at our institution. Of these 13 patients, 7 were managed with medical therapy alone, and 6 were referred for parathyroidectomy. The medical records were reviewed, and patients/relatives were interviewed. RESULTS: All patients had cutaneous wounds requiring local debridement predominantly located on the lower extremities or abdominal wall. Six patients underwent subtotal (3.5 gland) parathyroidectomy without morbidity. All 6 had significant reductions in parathyroid hormone levels after surgery (mean decrease, 94% +/- 0%), and all reported resolution of pain and healing of cutaneous wounds. Of the remaining 7 patients who had medical management alone, 5 eventually died of complications related to calciphylaxis. In comparing the 2 groups, patients who underwent parathyroidectomy had a significantly longer median survival than those who did not have surgery (36 vs 3 months, P =.021). CONCLUSIONS: Calciphylaxis frequently causes gangrene, sepsis, and eventual death. Parathyroidectomy can be performed with minimal morbidity and is associated with resolution of pain, wound healing, and a significantly longer median survival. Therefore, patients with secondary hyperparathyroidism and signs/symptoms of calciphylaxis should be referred promptly for consideration of parathyroidectomy.

Positron emission tomography in the detection and management of sarcomatous transformation in neurofibromatosis.

Benign neurofibromas undergo sarcomatous transformation in approximately 5% of patients with neurofibromatosis type I. The clinical and radiologic diagnosis of sarcomatous change remains difficult. Positron emission tomography with F-18 fluorodeoxyglucose is a method to assess increased glucose metabolism in malignant tissue such as sarcomas. In this case report, positron emission tomography accurately distinguished malignant from benign neurofibromas. The technique may be useful as a noninvasive screening tool for malignant transformation of neurofibromas.

Comparison of TriPath thin-layer technology with conventional methods on nongynecologic specimens.

OBJECTIVE: To evaluate the use of the TriPath PREP (previously called AutoCyte) TriPath Inc., Burlington, North Carolina, U.S.A.) in nongynecologic cytologic material by performing side-by-side comparison of conventional preparations with TriPath-prepared slides. STUDY DESIGN: An initial study of 613 cases (set A) was conducted to compare the TriPath PREP system with conventional methods for the evaluation of nongynecologic specimens, including urine, body cavity effusions, cerebrospinal fluid, pulmonary and gastrointestinal specimens. Paired cases were evaluated for cellularity, staining quality, preservation and representation of diagnostic material. Subsequent changes in the automated technique warranted reevaluation of the TriPath method. The follow-up study of 259 cases (set B) was conducted with the same design as set A. Results of evaluated parameters were analyzed using the chi 2 test. RESULTS: Results of the two sets were strikingly different. Prior to technical changes made by the laboratory, the TriPath method was significantly inferior. In the second set, the preferred material was most commonly the TriPath-prepared material. In particular, the majority of urine samples were prepared better by the automated, thin-layer system. CONCLUSION: The TriPath PREP system offers a reliable preparation of urine and has potential for other nongynecologic specimens, provided that careful attention is paid to technical details and some adjustments are made to account for specimen variability.

The accuracy of combined cytopathologic and flow cytometric analysis of fine-needle aspirates of lymph nodes.

We studied flow cytometry in 156 fine-needle aspirations (FNAs) of lymph nodes performed between June 1993 and September 1998. Information from flow cytometry was combined with cytomorphologic evaluation, and the diagnosis determined by using combined modalities was compared with tissue biopsy results or clinical follow-up. In 74 cases, a combined cytopathologic-flow cytometric diagnosis of lymphoma was made; histologic material was available for 52 patients; in no case was a benign process found. The lymphoma grade assigned agreed with histopathologic findings in 45 of 48 cases with a specific cytologic diagnosis. Treatment was initiated on the basis of the FNA alone for 17 of 52 patients with a history of lymphoma and in 22 additional patients with no follow-up biopsy. Among 71 cases in which the diagnosis using both modalities was benign, the only false-negative was 1 case of Hodgkin disease. Of the 156 cases, 11 were considered atypical or suggestive of lymphoma; biopsies from 8 of 10 patients revealed lymphoma. A combination of flow cytometry and cytomorphology of cells obtained by FNA of lymph nodes can distinguish between benign and malignant lymphoid infiltrates and support a diagnosis of lymphoma that permits definitive therapy in most cases.

Case report. Diagnosis of disseminated Mycobacterium avium complex infection by liver biopsy.

Mycobacterium avium complex (MAC) infection is a common complication of HIV/AIDS. Signs and symptoms of this infection are nonspecific and include fever, weight loss, diarrhea, and abnormal levels of liver enzymes, especially elevated alkaline phosphatase levels. Diagnosis can be achieved through several methods, but liver biopsy may be the most rapid and efficient. We present a case that illustrates the potential value of liver biopsy in diagnosing disseminated MAC infection.

Clinically significant, isolated metastatic disease to the thyroid gland.

Despite being second only to the adrenal glands in terms of relative vascular perfusion, the thyroid gland is a rare site of metastatic disease; but when thyroid metastases occur, long-term survival has been reported to be dismal. To determine the incidence and management of isolated, metastatic disease to the thyroid, we reviewed our clinical experience. Between June 1986 and August 1994 ten patients underwent thyroidectomy for isolated, metastatic disease of nonthyroidal origin (mean +/- SD age 58 +/- 6 years, 30% female). The primary tumors were renal cell carcinomas (RCCs) (n = 5), esophageal adenocarcinoma (n = 1), pulmonary squamous cell carcinoma (n = 1), gastric leiomyosarcoma (n = 1), lingual squamous cell carcinoma (n = 1), and parotid gland carcinoma (n = 1). Three patients underwent preoperative fine-needle aspiration (FNA), all of which were suggestive of metastatic disease. The mean time from resection of the primary tumor to thyroid metastases was 3.5 +/- 6.0 years (range 0-19.5 years). Total thyroidectomy (n = 5) or lobectomy (n = 5) was performed without morbidity or mortality. After a median follow-up of 5.2 years six patients are alive and two are free of disease. Moreover, no patients have had recurrent disease in the neck. Thus carcinomas metastatic to the thyroid represent a rare cause of clinically significant thyroid disease, with RCCs comprising 50%. Most thyroid metastases (80%) present within 3 years of primary tumor resection, but with RCC they can occur as late as 19 years. The diagnosis of metastatic disease should be suspected in patients with even a remote history of cancer, especially RCC, and an FNA revealing clear cell or spindle cell carcinoma. Contrary to previous reports, long-term survival can be achieved after resection of the metastatic tumor. Furthermore, thyroidectomy may also palliate/prevent the potential morbidity of tumor recurrence in the neck.

Progressive multifocal leukoencephalopathy demonstrating contrast enhancement on MRI and uptake of thallium-201: a case report.

We describe a patient with AIDS who presented with focal neurological symptoms, and who had contrast-enhancing brain lesions on MRI which demonstrated increased thallium-201 uptake on SPECT. These findings were consistent with lymphoma; however, brain biopsy established a diagnosis of progressive multifocal leukoencephalopathy (PML). To our knowledge, this is the first reported case of PML with increased thallium-201 uptake on brain SPECT.

Complete hepatic resection of metastases from leiomyosarcoma prolongs survival.

Although liver resection has been shown to prolong survival in selected patients with metastases from colorectal cancer, the benefit for other metastatic tumors is unproved. To determine whether hepatic resection has a role in the management of metastatic leiomyosarcoma, medical records from 11 consecutive patients who underwent resection of isolated metastases from leiomyosarcoma between 1984 and 1995 were reviewed. All liver resections were for leiomyosarcomas originating in the viscera (n = 6) or retroperitoneum (n = 5). The average disease-free interval was 16 months. Five of 11 primary tumors were classified as low grade, whereas six were high grade. Hepatic resections included lobectomy or extended lobectomy (n = 4), segmentectomy and/or wedge resection (n = 5), and complex resection (n = 2). There were no operative deaths. Median survival of all patients after liver resection was 39 months. Patients who underwent complete resection of hepatic metastases (n = 6) had a significantly longer survival than those who had incomplete resections (n = 5) (P = 0.03, log-rank test). Furthermore, five of six patients who underwent complete resection are alive after hepatectomy with a median follow-up of 53 months. Therefore, in selected patients with isolated liver metastases from visceral and retroperitoneal leiomyosarcomas, complete resection of hepatic metastases results in prolonged survival.

Metastatic testicular Sertoli-cell tumor: cytopathologic findings on fine-needle aspiration.

Malignant Sertoli-cell tumors of the testis are exceedingly rare. We present cytopathologic findings in pelvic metastasis of such a tumor in a 45-year-old man, diagnosed on fine-needle aspiration, 2 months after radical orchiectomy. Ultrasound-guided aspirate showed tissue fragments and isolated discohesive tumor cells with characteristics of testicular Sertoli cells. Immunoperoxidase (IPOX) studies and histopathologic correlation with the primary testicular tumor are also presented. Cytopathologic features complimented by IPOX studies should allow an accurate diagnosis of this rare entity, when seen at metastatic sites.

Intraabdominal desmoplastic small round cell tumor: cytopathologic findings in two cases.

Intraabdominal desmoplastic small round cell tumor (DSRCT) is an extremely rare entity. This study describes fine-needle aspiration, ascitic fluid, and touch imprint cytomorphology of DSRCT in 2 patients with extensive abdomino-pelvic disease. Cytopathologic features were unique and showed good morphologic correlation with subsequent histology of the resected tumor. Immunocytochemical profile and differential diagnosis with other small round cell tumors in this age group are also discussed.

Hürthle cell neoplasms of the thyroid: are there factors predictive of malignancy?

OBJECTIVE: To determine if any preoperative or intraoperative factors can reliably predict malignancy in patients with Hürthle cell neoplasms. SUMMARY BACKGROUND DATA: Most experienced surgeons recommend total thyroidectomy for Hürthle cell carcinomas and reserve thyroid lobectomy for Hürthle cell adenomas. However, delineation between Hürthle cell adenoma versus carcinoma often cannot reliably be made either before or during surgery. METHODS: Medical records from 57 consecutive patients who underwent thyroid resections for Hürthle cell neoplasms between October 1984 and April 1995 at The Johns Hopkins Hospital were analyzed to determine if any factors were predictive of malignancy. RESULTS: Of the 57 patients with Hürthle cell neoplasms, 37 had adenomas and 20 had carcinomas, resulting in a 35% prevalence of malignancy. Patients with adenomas did not differ from those with carcinoma with respect to age, sex, or history of head and neck irradiation. However, patients with Hürthle cell carcinomas had significantly larger tumors (4.0 +/- 0.4 cm vs. 2.4 +/- 0.2 cm, p < 0.005). Furthermore, although the incidence of malignancy was only 17% for tumors 1 cm or less and 23% for tumors 1 to 4 cm, tumors 4 cm or greater were malignant 65% of the time (p < 0.05). Both fine-needle aspiration and intraoperative frozen section analysis had low sensitivities in the detection of cancer (16% and 23%, respectively). With up to 9 years of follow-up, there has been no tumor-related mortality. CONCLUSIONS: These data demonstrate that the size of a Hürthle cell neoplasm is predictive of malignancy. Therefore, at the time of initial exploration for large Hürthle cell neoplasms (>4 cm), definitive resection involving both thyroid lobes should be considered because of the higher probability of malignancy.

Granulocytic sarcoma (chloroma) of the sacrum: initial manifestation of leukemia.

We present an unusual case of a granulocytic sarcoma (chloroma) of the sacrum which predated the initial clinical manifestation of acute myelogenous leukemia. Although granulocytic sarcomas occur in up to 9.1% of cases of acute myelogenous leukemia they usually present concurrently with the leukemic presentation. Although granulocytic sarcomas can involve several different organ systems, bone is the most common site.

Follicular lesions of the thyroid. Does frozen section evaluation alter operative management?

OBJECTIVE: The authors determined the usefulness of frozen section (FS) evaluation in the operative management of follicular lesions of the thyroid. SUMMARY BACKGROUND DATA: Fine-needle aspiration (FNA) cannot reliably discriminate between benign and malignant follicular lesions of the thyroid. Accordingly, FS evaluation is used routinely to guide intraoperative management. METHODS: One hundred twenty-five consecutive patients with follicular thyroid lesions who underwent surgical exploration at the Johns Hopkins Hospital were reviewed. RESULTS: Frozen sections were categorized in 104 of 120 patients (87%) as "follicular lesion, defer to permanent section," rendering no useful clinical information. In only 4 of 120 patients (3.3%) did FS evaluation correctly modify the operative procedure. Notably, in six cases (5.0%), an incorrect FS evaluation misled the surgeon, resulting in four misguided operations. CONCLUSION: Frozen section evaluation is of minimal diagnostic value for follicular thyroid lesions, rendering no additional information 87% of the time; it prolongs the operation, increases costs, and leads to misguided interventions. Until a more definitive diagnostic tool exists for follicular thyroid lesions, FS evaluation could be omitted, resection of the lobe with the nodule could be performed, and the definitive operative management could be based on the final permanent histology.

Intraairway Feulgen-positive bodies in hyaline membrane disease of the newborn.

In reviewing cases of hyaline membrane disease of the newborn (HMD) we have been struck by the occurrence of dense basophilic bodies within the airways. To further examine this phenomenon we reviewed lung histology from 246 consecutive stillborns and liveborns 24 hours or less of age autopsied at The Johns Hopkins Hospital. Cases with autolysis or major malformations were excluded. We found the basophilic bodies in 61 (71%) of 87 infants with HMD. The material forming the bodies was Feulgen-positive and derived from necrotic respiratory epithelial cells. Pyknotic nuclei were extruded from necrotic cells and fused to form the bodies some of which reached a size of over 50 microns in maximum dimension. Among the 159 cases without HMD, only 13, all of which were among 81 cases of fetal pneumonia, showed small, 2-5 microns in diameter, intraairway Feulgen-positive bodies. The bodies in these cases of fetal pneumonia were derived from disintegrating leukocytes. The study shows that the formation of intraairway Feulgen-positive bodies derived from necrotic epithelial cells is a common finding in infants dying with HMD during the first day of life. The early development and frequent occurrence of the bodies lends support to the idea that respiratory epithelial cell necrosis is the cause of HMD of the newborn.