Endometriotic Mass After Hysterectomy in a 61 Year Old Post-menopausal Woman: A Case Report and Update.

Endometriosis is a common, hormone-dependent gynecologic disease. Undiagnosed in large proportion of women, managing therapies depend on the impact of quality of life and includes hormonal treatment and pelvic surgery. Less likely endometriosis can occur in post-menopausal women. Malignant transformation of endometriosis is a rare but well-described process, most of time occurring in the ovary, and justifies the practitioner not to underestimate this pathology. We present a case of a 61 year old woman with a symptomatic endometriotic pelvic mass, status post hysterectomy, with no history of endometriosis diagnosed beforehand.

Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients.

PURPOSE: To identify most significant and therapeutically relevant prognostic factors in adults with localized primary synovial sarcomas (SS) and to confirm the usefulness of the French Federation of Cancer Centers (FNCLCC) grading system, the prognostic impact of which has been already proven in soft tissue sarcomas. PATIENTS AND METHODS: Data on 128 patients with nonmetastatic SS collected from a cooperative database by the FNCLCC Sarcoma Group between 1980 and 1994 were studied retrospectively. Immunohistochemistry was performed at diagnosis in 77 cases (61%). The tumors were classified as biphasic (n = 45), monophasic fibrous (n = 72), and poorly differentiated (n = 10) subtypes. Histologic grade was determined according to the FNCLCC method, and vascular invasion was assessed in every case. RESULTS: The 5-year disease-specific survival (DSS) rate for this series of patients with localized SS was 62.9% (+/- 9.6% [SD]) with a median follow-up time of 37 months (range, 8 to 141 months). In multivariate analysis, the adverse risk factors associated with decreased DSS were International Union Against Cancer/American Joint Committee on Cancer stage III/IVA disease, male sex, and truncal tumor locations. For metastasis-free survival (MFS), disease stage III/IVA, tumor necrosis, and monophasic subtypes were the major factors associated with a less favorable prognosis. Separately, when not using disease stage, tumor necrosis, and mitotic activity, histologic grade became the most significant prognostic factor for both DSS and MFS. In addition, larger tumors and older patients become associated with a significantly worse prognosis. Independent adverse risk factors for local recurrence-free survival included histologic grade 3 and truncal tumor location. CONCLUSION: These data confirm that not all SS present the same severe outcome. High-risk patients identified on the basis of these parameters may qualify for an aggressive treatment approach.

Immunohistology of carcinoembryonic antigen (CEA)-expressing tumors grafted in nude mice after radioimmunotherapy with 131I-labeled bivalent hapten and anti-CEA x antihapten bispecific antibody.

We have developed a pretargeting strategy, called the Affinity Enhancement System (AES), which uses bispecific antibodies (BsF(ab')2) to target radiolabeled bivalent haptens to tumor cells. We performed several radioimmunotherapy (RIT) experiments in nude mice grafted with LS174T colon carcinoma or TT medullary thyroid cancer. Mice were treated with 131I-labeled di-DTPA-indium-tyrosyl-lysine bivalent hapten (75-112 MBq) administered 15-48 h after anti-CEA x anti-DTPA-indium BsF(ab')2. Immunohistological studies were performed on tumors at their minimal relative volume (TT), on stabilized tumor nodules (LS174T), and on regrowing tumors (TT and LS174T). Untreated tumors were used as controls. On microscopic examination, regrowing tumors (2 months posttherapy) were similar to untreated tumors with cells showing their respective typical morphology (large cells with a high nucleocytoplasmic ratio for TT, small and very undifferentiated cells for LS174T). However, regrowing tumors showed larger necrotic areas and a higher mitotic index correlated with Ki-67 antigen staining. Immunostaining for CEA was as strong as for controls. By contrast, the immunohistology of TT tumors at their minimal relative volume (1 month posttherapy) or of LS174T residual nodules (8 months posttherapy) showed decreased mitotic indices correlated with poor Ki-67 antigen staining. Some clusters of LS174T presented with features of glandular lumen, which suggested a more differentiated and less aggressive status. In TT tumors, CEA expression remained unchanged (80-100% membrane and cytoplasmic staining), whereas only 70% of the LS174T tumors were stained, with 58% loss of the membrane expression. Repeated treatment early after the tumor has reached its minimal relative volume should thus be efficient and improve the overall efficacy of AES RIT.

Toxicity and efficacy of radioimmunotherapy in carcinoembryonic antigen-producing medullary thyroid cancer xenograft: comparison of iodine 131-labeled F(ab')2 and pretargeted bivalent hapten and evaluation of repeated injections.

This study compared the toxicity and efficacy of 131I-labeled bivalent hapten pretargeted by anti-carcinoembryonic antigen (CEA)/anti-N alpha-(diethylenetriamine-N,N,N',N''-tetraacetic acid-indium(F6-734) bispecific antibody [affinity enhancement system (AES) reagents] with 131I-labeled anti-CEA F(ab')2 (131I-F6) in mice grafted with a human medullary thyroid carcinoma. Repeated injections of AES reagents were also evaluated. Mice bearing TT tumor xenografts were treated with 37, 74, or 92.5 MBq of AES reagents, two injections of 74 MBq of AES reagents 45 days apart, or 37 or 92.5 MBq of 131I-F6. Control groups were treated with nonspecific 131I-labeled F(ab')2, nonspecific AES reagents, nonradiolabeled F6, F6-734 bispecific antibody, and nonradiolabeled bivalent hapten or received no injection. For AES treatments, bispecific antibody was injected 48 h before the hapten. Animal weight, hematological toxicity, tumor volume, and serum thyrocalcitonin were monitored during 5 months. At 92.5 MBq, weight loss was significantly lower after AES than F6 treatment (P = 0.004). The percentages of leukocyte count changes were significantly lower after AES than F6 at 37 and 92.5 MBq (P = 0.01 and 0.04, respectively). The percentage of platelet count changes was significantly lower with AES at the 92.5-MBq dose level (P = 0.04). In the group injected twice with AES reagents, toxicity was not significantly increased after the second treatment. Tumor response was observed in all cases but was significantly longer with repeated treatments of 74 MBq AES reagents than with a single treatment (P = 0.004). Two complete responses were observed with repeated treatments. Changes in thyrocacitonin level paralleled those in tumor volume. These results indicate that pretargeted radioimmunotherapy was at least as efficient as one-step radioimmunotherapy and markedly less toxic. Repeated treatments with AES reagents increased efficacy without increasing toxicity.

Adenovirus-mediated gene transfer into isolated mouse adult pancreatic islets: normal beta-cell function despite induction of an anti-adenovirus immune response.

The in vitro purification of pancreatic islets offers an opportunity for their modification by ex vivo gene transfer. We investigated the efficiency and functional consequences of adenovirus-mediated gene transfer into adult murine pancreatic islets with a recombinant adenovirus encoding for the beta-galactosidase (beta-Gal) reporter gene. At 10(6) pfu/islet, almost all of the islets were transduced, but maximal transduction was obtained at 10(7) pfu/islet. Histochemical analysis of frozen islet sections showed that transduced cells were only located at the periphery of islets. Transduced islets showed normal insulin secretion in vitro, and were able to normalize in vivo the glycemia of streptozotocin-induced diabetes in syngeneic and allogeneic mice. beta-Gal expression in transduced islets was observed for at least 6 weeks in naive normal recipients and in immunodeficient mice, but was shortened in mice preimmunized to adenovirus. Nevertheless, islets maintained normal control of glycemia in all mice. An early leukocyte infiltrate was observed in syngeneic grafts of transduced islets, but no acceleration in rejection of fully MHC-incompatible islet grafts occurred. In summary, adenovirus-mediated gene transfer in adult mouse islets, although sparing most of the beta-cells, was highly efficient and did not impair insulin secretion by islets. The immune response to the adenovirus and/or to the transgene might be only partially responsible for the decreased expression over time of the transduced gene. Accordingly, adenovirus-mediated gene transfer might allow efficient expression of vectorized sequences with potential immunosuppressive effects in the islet microenvironment.

Biodistribution and dosimetric study in medullary thyroid cancer xenograft using bispecific antibody and iodine-125-labeled bivalent hapten.

UNLABELLED: The purpose of this study was to evaluate biodistributions and absorbed doses of anti-carcinoembryonic antigen (CEA)/anti-diethylenetriamine pentaacetic acid (DTPA)-indium (anti-DTPA-In) bispecific monoclonal antibody (BsMAb) F6-734 and 125I-labeled DTPA-indium dimer hapten (125I-di-DTPA-In hapten) in athymic mice xenografted with human medullary thyroid cancer. METHODS: Bispecific monoclonal antibodies F6-679 (anti-CEA/antihistamine) and G7A5-734 (antimelanoma/anti-di-DTPA-In) were used as irrelevant BsMAbs. Athymic mice inoculated with TT medullary thyroid cancer cells expressing CEA were administered BsMAbs F6-734, F6-679 or G7A5-734 and then, 48 hr later, 125I-di-DTPA-In hapten. Iodine-125-labeled F6 F(ab')2 fragment was injected into other groups of mice. Biodistributions were examined at 30 min and 5, 24, 48 and 96 hr after injection of 125I-di-DTPA-In hapten or 125I-labeled F6 F(ab')2. RESULTS: In mice injected with BsMAb F6-734 and 125I-di-DTPA-In hapten, tumor uptake was 9.1%+/-2.1%, 8.7%+/-3.5%, 8.0%+/-2.3%, 5.1%+/-0.9% and 3.5%+/-1.5% of the injected dose/g at 30 min and 5, 24, 48 and 96 hr, and tumor-to-blood, tumor-to-liver and tumor-to-kidney ratios were 37.0+/-12.5, 32.3+/-10.9 and 10.4+/-2.7 at 24 hr. Iodine-125-F6 F(ab')2 fragment showed a tumor uptake of 7.39% injected dose/g and tumor-to-blood, tumor-to-liver and tumor-to-kidney ratios of 1.8+/-0.6, 7.3+/-2.9 and 3.6+/-1.6 at 24 hr. In mice injected with F6-679 or G7A5-734, tumor uptake and tumor-to-normal tissue ratios were much lower than in the mice injected with F6-734. These results were confirmed by autoradiographic studies that demonstrated clear tumor-to-normal tissue contrast. CONCLUSION: This two-step targeting method seems very potent for the diagnosis and therapy of human medullary thyroid cancer and other CEA-producing tumors because it combines high tumor uptake and low normal tissue background.

Bispecific antibody and bivalent hapten radioimmunotherapy in CEA-producing medullary thyroid cancer xenograft.

UNLABELLED: The purpose of this study was to compare the toxicity and efficacy of two-step radioimmunotherapy using a bispecific anticarcinoembryonic antigen (CEA)/anti-diethylenetriamine pentaacetic acid (DTPA) antibody (F6-734 bispecific monoclonal antibodies (BsMAbs) and an 131I-di-DTPA-TL bivalent hapten with F(ab')2 fragments of the same directly labeled anti-CEA 131-F6. METHODS: Eight groups of nude mice subcutaneously grafted with the human TT medullary thyroid cancer cell line were injected once tumor volume reached about 200 mm3. Two groups received 37 or 92.5 MBq (1 or 2 nmol) 131I-di-DTPA-TL 48 h after injection of 2 or 4 nmol F6-734 BsMAb and two groups received 37 or 92.5 MBq (250 microg) 131I-F6. Four control groups were treated respectively with (a) 92.5 MBq nonspecific 131I-734 fragments, (b) 92.5 MBq 131I-di-DTPA-TL 48 h after injection of a mixture of irrelevant F6-679 (anti-CEA/anti-histamine) and G7A5-734 (anti-melanoma/anti-DTPA) BsMAb, (c) 250 microg nonradiolabeled F6, and 250 microg F6-734 BsMAb and then 48 h later 1.25 nmol of nonradiolabeled hapten. A control group received no injections. Toxicity was evaluated by determining animal weight and the number of leukocytes and platelets, and efficacy by variation in tumor volume and thyrocalcitonin during a 90-d period. Histological analysis of tumors and statistical studies were performed. RESULTS: The time required for the tumor to double in size was respectively 57 and 86 d with 37 and 92.5 MBq F6-734/131I-di-DTPA-TL and 44 and 65 d with 37 and 92.5 MBq 131I-F6. Changes in thyrocalcitonin levels were parallel to those in tumor volume. Weight loss was 5%, leukocyte nadirs respectively 1640+/-838 and 1560+/-1160/mm3 and platelet nadirs 1.46+/-0.52 10(6)/mm3 and 0.73+/-0.38 10(6)/mm3 after injections of 37 and 92.5 MBq F6-734/1311-di-DTPA-TL. Weight loss was respectively 8% and 16%, leukocyte nadirs 50+/-100/mm3 and 175+/-50/mm3 and platelet nadirs 0.71+/-0.18 10(6)/mm3 and 0.48+/-0.11 10(6)/mm3 after injections of 37 and 92.5 MBq 131I-F6. CONCLUSION: Two-step radioimmunotherapy was as efficient as the one-step system and markedly less toxic.

Bispecific antibody and iodine-131-labeled bivalent hapten dosimetry in patients with medullary thyroid or small-cell lung cancer.

UNLABELLED: The purpose of this study was to estimate the dose delivered to tumor targets and normal tissues after two-step injection of an anti-CEA/anti-DTPA-In (F6-734) bispecific antibody and a 131I-labeled di-DTPA in-TL bivalent hapten in patients with medullary thyroid carcinoma (MTC) and small-cell lung cancer (SCLC). METHODS: Five patients with persistent disease or recurrences of MTC and five patients with primary SCLC or relapse were studied. In a first step, 0.1 to 0.3 mg/kg of F6-734 bispecific antibody was injected intravenously. Four days later, 6 nmole (5.8 to 9.8 mCi) of 131I-labeled di-DTPA in-TL bivalent hapten were injected. Quantitative imaging was performed during one week after the second injection. RESULTS: All 5 patients with MTC showed positive immunoscintigraphy (IS). In the smallest visualized and resected tumor (0.8 g), the fraction of injected activity per gram (% ID/g) was 0.1% at Day 3. IS was positive in 4 of the 5 patients with SCLC. The volume of the smallest visualized SCLC tumor was estimated at 11 +/- 2 ml, and tumor uptake was about 0.009% ID/g. Tumor dose estimates ranged from 4.2 to 174 cGy/mCi in patients with MTC and from 1.7 to 8 cGy/mCi in patients with SCLC. CONCLUSION: High absorbed dose values were calculated for small MTC recurrences. For SCLC recurrences the values were smaller but in the same range as those obtained by other investigators with the one-step technique in lymphoma.

Synchronous pulmonary adenocarcinoma and extranodal marginal zone/low-grade B-cell lymphoma of MALT type.

A case of synchronous adenocarcinoma of lung and extranodal marginal zone/low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is reported. Primary pulmonary non-Hodgkin's lymphoma is relatively rare, however, the majority of these lesions are low-grade B-cell lymphomas of MALT. After the stomach, the lung is the second most common location for such latter lesions. Lung adenocarcinoma in selected countries is fast becoming the leading form of non small-cell lung carcinoma. To our knowledge, this synchronous occurrence in the lung has not been previously reported. Such associations have been primarily limited to gastric lesions where an association with Helicobacter pylori infection has been identified. This case report highlights the importance of adjunctive diagnostic investigations such as molecular techniques in conclusive analysis of synchronous cases such as ours.

Primary Epstein-Barr virus infection with clonal T-cell lymphoproliferation.

A case of fatal Epstein-Barr virus infection in a previously healthy girl who was first found to have severe infectious mononucleosis with spontaneous recovery is reported. Because an abnormal immune response to the virus persisted, the disease relapsed, manifesting in cutaneous and pulmonary lesions associated with hemophagocytic syndrome responsible for death. Pathologic findings were characterized by polymorphous atypical lymphoid infiltrate, prominent necrosis, and histiocytic hyperplasia. Lymphoid cells displayed CD8 phenotype and clonal T-cell receptor gene rearrangement. Viral genome was detected in lesions by Southern blot and located in nuclei of lymphoid cells by in situ hybridization. Pathologic findings suggested fatal infectious mononucleosis; however, phenotype and genotype favored a malignant diagnosis. Clonality was demonstrated to have arisen during primary infection. Virologic examination indicated that Epstein-Barr virus was a causative agent. Such a process belongs to the recently recognized spectrum of Epstein-Barr virus-related T-cell lymphoproliferative disorders that might overlap fatal infectious mononucleosis in patients who are especially vulnerable to the virus.

Learning curve for the detection of axillary sentinel lymph node in breast cancer.

AIM: Sentinel axillary lymph node (SALN) detection is a new technique. Surgeons must progress up a learning curve in order to guarantee quality and safety equivalent to axillary lymphadenectomy. To ensure accurate staging of patients this learning curve must include SALN detection and an axillary lymphadenectomy. The aim of our work was to validate the principles and evaluate the consequences of learning curve for SALN detection from a prospective series of 200 consecutive patients. METHOD: Prospective assessment was made of the detection and false negative rates, post operative morbidity as abcess and seroma, and length of hospital stay. RESULTS: We evaluated the performance from the first to the hundredth case for each surgeon. Detection rate improved to 85% after patient number 10. False negative rate was less than 6%. Post operative axillary morbidity included 11% of seromas and 2% of abcess. Mean hospital stay was 2.8 days. CONCLUSION: Multidisciplinary validation of the learning period contributes to an accurate and safe SALN.

[Standards, Options and Recommendations (SOR) for drafting of anatomic and surgical pathology reports or cytopathology reports in oncology]. / Standards, Options et Recommandations (SOR) pour la rédaction d'un compte rendu d'anatomie et de cytologie pathologiques en cancérologie.

CONTEXT: The "Standards, Options and Recommendations" (SOR) project, started in 1993, is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery. OBJECTIVES: To develop practice guidelines according to the definitions of the Standards, Options and Recommendations project for the content of the anatomic and surgical pathology or cytopathology reports in field of oncology. METHODS: Data were identified either by searching on Medline or via members of the expert groups personal references lists. When the guidelines were defined, the document was submitted to 49 independent reviewers, and to the medical committees of the 20 French Cancer Centres. RESULTS: The main recommendations for the drafting of the anatomic and surgical pathology or cytopathology reports in oncology are: 1) The reports must contain the identification of the pathologist, of the patient and of the specimen, a gross description for the surgical specimen, eventually a microscopic description, the diagnosis, all the elements essential for establishing the prognosis and for the clinical care, and a conclusion. 2) The reports could contain some comments. 3) The reports must be brief, precise, clear, homogeneous and ideally standardised, in order to be comprehensible for all the clinicians and the pathologists.

[Right atrioventricular metastasis of myxoid liposarcoma. Prolonged course after repeated surgery and chemotherapy]. / Métastase auriculo-ventriculaire droite d'un liposarcome myxoïde. Evolution prolongée avec chirurgie itérative et chimiothérapie.

The authors report a case of cardiac metastasis of a myxoid liposarcoma of the thigh in a 54 year old woman. The treatment associated repeated surgical ablation and chemotherapy. The drugs used induced some cardiotoxicity which limited their prescription but nevertheless, the patient survived 5 years. Previous reported cases do not describe survival lasting more than 2 years and associated chemotherapy would therefore seem to be a valuable adjuvant, increasing the life expectancy of such patients.

[Extension of an acinar cell pancreatic carcinoma with cystic changes invading the Wirsung canal]. / Carcinome à cellules acineuses pancréatique d'allure kystique, envahissant le canal de Wirsung.

We report a case of acinar cell carcinoma of the pancreas with misleading cystic changes. A 32-year-old woman presented with symptoms suggesting acute pancreatitis on chronic pancreatitis. The abdominal computed tomography and the endoscopic retrograde pancreatography demonstrated hypertrophy of the pancreatic head associated with global dilatation of main pancreatic duct and secondary canals and a 5 cm communicating cyst. A intraductal papillary-mucinous tumor was suggested. Microscopic findings showed a poorly differentiated adenocarcinoma. Six months later, a liver metastasis was detected. The microscopic appearance was different, suggesting acinar cell carcinoma, confirmed by immunohistochemistry. Only two other cases of acinar cell carcinoma with cystic component have been reported in the literature.

[Primary granulocytic sarcoma of the pancreas: efficacy of early treatment with intensive chemotherapy]. / Sarcome granulocytique primitif du pancréas: efficacité d'un traitement précoce par chimiothérapie intensive.

In the absence of systemic chemotherapy after the diagnosis of primary granulocytic sarcoma (PGS), all patients will subsequently develop acute myelogenous leukemia (AML). The authors describe a case of PGS of the head of the pancreas found at laparotomy in a 32-year-old man. The patient received early after surgery, two courses of high-dose chemotherapy and, with a follow-up of 2 years, never developed AML. This rare observation illustrates the necessity of further early systemic chemotherapy after surgical excision of PGS.

[Neuroendocrine tumor of the larynx. Report of a case]. / Tumeur neuro-endocrine du larynx. A propos d'un cas.

Neuro-endocrine tumors of the larynx are uncommon. This paper reports a case of well differentiated neuro-endocrine carcinoma of the epiglottis of a 61-year-old man. Three years later, he developed a cervical larynx node and four years later, metastatic nodules on his scalp. The tumor produced calcitonin. Serotonin and metenkephalin were also secreted in skin metastases. Neuro-endocrine tumors of the larynx have a bad prognosis. Wenig proposed a classification adaptable to laryngeal neuroendocrine carcinoma based on histologic features and behavior.

[Diffuse sclerosing papillary carcinoma of the thyroid. Report of a case]. / Carcinome papillaire sclérosant diffus de la thyroide. Présentation d'un cas.

The authors report the case of a 12-year-old girl presenting with a diffuse sclerosing papillary carcinoma of the thyroid. The diagnosis was made on a total thyroidectomy specimen three months after thyroid enlargement was detected. This child had previously been treated medically for thyroiditis because of an enlarged thyroid gland with serum antithyroid autoantibodies. A few cases have been described in the literature. Six morphologic findings define this variant of papillary carcinoma: a diffuse growth pattern involving one or both lobes of the thyroid, prominent fibrosis, heavy lymphocytic infiltration with germinal centers, large numbers of psammoma bodies, squamous metaplasia and papillae within cleft-like tissue spaces. Some authors stated that this variant has a poorer prognosis than the usual papillary carcinoma of the thyroid. However, in some series a few patients survive without local recurrence or distant metastasis at a mean follow-up period of more than 10 years. Immunohistochemical studies have shown high accumulation of S-100 protein positive dendritic cells. Such an infiltration has been correlated with a better prognosis.

["Mixed' (follicular and parafollicular) carcinomas of the thyroid. Histological and immunocytological study of 5 cases]. / Les carcinomes "mixtes" (vésiculaires et paravésiculaires) de la thyroïde. Etude histologique et immunocytochimique de 5 cas.

We studied five cases of poorly differentiated follicular or papillary thyroid carcinomas. Immunohistochemical study revealed numerous ACE positive cells, also positive for calcitonin, ACTH, somatostatin or several of these peptides. These tumors containing both vesicular component and parafollicular cells are endocrine tumors of "mixed" or "intermediate" type. The diagnosis must be confirmed by immunohistochemistry but can be suggested by histological findings: abundant fibrous stroma, trabeculovesicular pattern, and swelled moderately acidophilic cells neighbouring vesicular cells. These facts argue in favor of a common-embryological origin of vesicular and parafollicular cells from ultimobranchial undifferentiated cells. Nevertheless such tumors must take place in thyroid neoplasia's classifications and an appropriate terminology remains to be precised.

[Breast metastasis from a "tall cell variant" of papillary thyroid carcinoma]. / Métastase mammaire d'un carcinome papillaire à cellules hautes de la thyroïde.

The authors report a case of breast metastasis from a "tall cell" variant of papillary thyroid carcinoma in a 59-year-old woman. This metastasis was discovered two months after the diagnosis of an inextirpable thyroid tumor. Tall cell variant of papillary thyroid carcinoma is a sub-type of papillary carcinoma with an aggressive course and frequent metastases (in patients over 50 years of age). No mammary metastasis of this tumor type has been reported so far.

[Immunohistochemical study of 6 multiple familial cervical paragangliomas with lymph node metastasis in one case]. / Etude immunohistochimique de six paragangliomes cervicaux multiples et familiaux avec métastase ganglionnaire dans un cas.

Cervical localizations of extra-adrenal paragangliomas are infrequently malignant, especially in their familial forms. An immunohistochemical study was performed on 6 cervical paragangliomas which were detected in 3 sisters at the age of 20 20. One had a lymph node metastasis. This study confirmed the diagnosis of paraganglioma with endocrine chief cells and supratentacular cells. It also enabled the hormonal contents of these cervical paraganglioma to be determined. In addition to catecholamine, these tumors may (like pheochromocytomas) produce serotonin and one or more other peptides.