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Biomedical subjects

S T Pierce

Publications and source records attributed to S T Pierce.

8 recordsLinked to original sources

Long-term survival in refractory recurrent neuroblastoma.

BACKGROUND: Patients older than age 1 year with metastatic neuroblastoma have poor survival. A patient with a unique history of metastatic neuroblastoma by virtue of disseminated nodal disease with multiple relapses and a 14-year survival is presented. METHODS: A case history of a 24-year-old man who was diagnosed at age 9 years with Evans stage IV neuroblastoma with multiple recurrences is reviewed. A review of the literature was undertaken to uncover similar patients with advanced stage disease and an absence of hematogenous metastasis. RESULTS: Eleven patients older than age 1 year, in addition to the case presented, have been reported in the literature with stage IV disease without extralymphatic metastasis. These patients have a survival of 50% at 5 years as compared with 15% in historical controls. Long-term survival with recurrent disease has not previously been noted in this population. Other favorable prognostic factors including favorable histology, and a single N-myc oncogene copy were documented in the present case. CONCLUSION: A tendency toward improved survival is seen in patients with nonhematogeneous stage IV neuroblastoma. It is unknown whether long-term survival with residual disease, as observed in the present case, is seen in other patients with stage IV disease without extralymphatic metastasis. Prospective analysis of other patients with stage IV neuroblastoma without extralymphatic metastasis is required to confirm these observations.

Adolescent↗

Myeloid markers in adult acute lymphocytic leukemia. Correlations with patient and disease characteristics and with prognosis.

BACKGROUND: The expression of myeloid markers on lymphoblasts has been associated with adverse outcome in acute lymphocytic leukemia (ALL). The purpose of the study was to analyze the experience with adults treated at the University of Texas M. D. Anderson Cancer Center with myeloid marker- (MY) positive ALL in relation to patient and disease characteristics, response to therapy, and prognosis. METHODS: Since 1988, 64 of 162 adults (40%) with newly diagnosed ALL referred to our service had MY-positive ALL. Their characteristics and outcomes were compared with the 98 patients with MY-negative ALL. Patients were treated with the vincristine-doxorubicin-dexamethasone (VAD) regimens. RESULTS: Patients with MY-positive ALL were significantly older (median ages, 47 years vs. 33 years; P = 0.03), had a higher incidence of CD34 antigen expression (59% vs. 36%; P < 0.01), and a lower incidence of common acute leukemia antigen expression (50% vs. 71%; P < 0.01), serum alkaline phosphatase elevation (58% vs. 83%; P < 0.01), and thrombocytopenia at diagnosis (49% vs. 69%; P = 0.02). Myeloid marker positivity, as expected, was significantly higher in null cell ALL (82%), and significantly lower in mature B-cell ALL (17%) (P < 0.01). Forty-one of 64 MY-positive patients achieved complete remission (CR) after induction therapy compared with 76 of 98 patients MY-negative disease (CR rate 64% vs. 78%; P = 0.06). With a median follow-up of 45 months, no statistical differences were observed in remission duration or survival between MY-positive and MY-negative patients, overall, and within immunophenotypic subsets (T-cell vs. others), or among subgroups with single marker (CD13, CD14, CD33, CD34) positivity. The 3-year remission duration rates were 32% for MY-negative and 40% for MY-positive patients (P not significant), and 3-year survival rates were 26% and 31%, respectively (P not significant). CONCLUSIONS: With VAD therapy, myeloid marker positivity is not associated with significant differences in prognosis in adult ALL.

Adolescent↗

Malignant joint effusions secondary to solid tumor metastasis.

We describe the case of a patient with breast cancer who developed bilateral malignant knee effusions, and review the English language literature. Patients with solid tumors who develop malignant joint effusions are rare; 27 cases have been reported (including our patient). The knee is the predominant site of joint involvement (p < 0.001). Synovial fluid (SF) analysis often demonstrates noninflammatory bloody effusion. Synovial biopsy was positive in 11 of 16 cases in which it was performed. SF cytology was positive in one half of cases in which it was obtained. Arthritis secondary to metastasis is a poor prognostic finding. A strong clinical suspicion is necessary to make the diagnosis; SF cytology or synovial biopsy can confirm it.

Bone Neoplasms↗

Paraendocrine syndromes.

Paraendocrine syndromes are the remote effects of cancer that result from ectopic hormonal production. Recent investigations have concentrated in five areas that include the evaluation of diagnostic studies, the recognition of rare cases, the contribution of secondary hormones, the clinical distinctions of ectopic syndromes from endocrine disorders, and the efficacy of pharmaceutical agents in correcting hormonally induced biochemical imbalances. The three most common paraendocrine syndromes (humoral hypercalcemia of malignancy, the syndrome of inappropriate antidiuretic hormone production, and ectopic Cushing's syndrome) are the subject of this review.

Cushing Syndrome↗

Rhabdomyolysis associated with the use of intravenous vasopressin.

A patient with acute esophageal variceal bleeding developed fatal rhabdomyolysis during treatment with a continuous intravenous infusion of vasopressin. Signs of ischemia, including mottling of skin and painful extremities, preceded the development of the characteristic electrolyte abnormalities and cardiac arrhythmias. No other recognized causes of rhabdomyolysis were identified on retrospective review of the hospital course. There are several factors which might promote a peripheral ischemic response to vasopressin in the bleeding cirrhotic patient, including altered resting hemodynamics, increased resting sympathetic tone, impaired vasodilation as a compensatory response to vasopressin, and reduced hepatic drug clearance. Idiosyncratic factors involving vasopressin receptor affinity and distribution, vasopressin-associated vasodilation in some vascular beds, and the effect of vasopressin on the renin-angiotensin system may further contribute to impaired tissue perfusion. These multiple overlapping factors probably lead to rhabdomyolysis in a minority of patients receiving vasopressin infusion.

Aged↗

Small cell carcinoma with two paraendocrine syndromes.

Simultaneous elevated levels of ectopic arginine vasopressin (AVP) and ectopic adrenocorticotropin (ACTH) were found in a patient with small cell carcinoma (SCC). The finding of one of these paraendocrine syndromes at the time of diagnosis is common; however, the simultaneous presence of both syndromes has been reported in the literature only on four occasions in the past 25 years. This is the only report in which elevated plasma levels of both hormones are documented in a patient who simultaneously fulfills the criteria for the syndrome associated with each ectopically produced peptide. In the English-language literature, this is the first case that demonstrates by immunohistochemical staining the presence of both of these hormones in the patient's neoplasm. In addition to the use of radiographs, the presence of paraendocrine disorders can provide a method of monitoring the patient's response to therapy. The levels of ACTH and AVP were assayed during this patient's course and correlated with disease refractory to therapy, resulting in poor survival.

ACTH Syndrome, Ectopic↗

Developmental patterns of neurite outgrowth from chick embryo spinal cord and retinal neurons on laminin substrates.

It has been previously found that neurite outgrowth on collagen substrates decreases with increasing gestational age of chick embryo spinal cord and retinal neurons in tissue culture. In the current study, laminin, polylysine and collagen were compared in their efficacy in promoting neurite extension from chick embryo spinal cord neurons aged 6-16 days or retinal neurons aged 8-16 days in ovo. The percentage of neurons with neurites and the length of the neurites were determined at 1 and 3 days in culture. There was a significant increase in neuritogenesis by laminin and polylysine compared to collagen for both spinal cord and retinal neurons. Further, in spinal cord cultures grown on a laminin substrate, there was no decline in neurite outgrowth with increasing developmental age of the neurons as was seen on collagen and polylysine. Neurite length measurements also demonstrated a significant stimulation of neuritogenesis for spinal cord, but not retinal, neurons by laminin compared to polylysine or collagen in 1-day cultures. The results demonstrate tissue-specific differences in the developmental patterns of neurite outgrowth. Retinal neurons appear to have intrinsic changes in their ability to respond to extracellular promoting factors or substrates, while spinal cord neurite outgrowth can be regulated by these extrinsic factors.

Animals↗

Bone marrow metastases from small cell cancer of the head and neck.

BACKGROUND: Primary small cell carcinoma of the head and neck is rare. Although the larynx is the most prevalent site of head and neck small cell carcinoma (SCC), this report will concentrate on SCC of the major salivary glands and paranasal sinuses. In all, 33 cases of paranasal sinus and 43 cases of major salivary gland SCC have been reported in the literature. METHODS: We report two patients, one with submandibular gland SCC and the other with maxillary sinus SCC. A literature review of all known paranasal sinus and major salivary gland SCC with inclusion of data from these two new cases is undertaken. Discussion of all past and present cases concentrates on sites of metastasis, treatment, and survival. RESULTS: Paranasal sinus SCCs predominantly arise from the nasal cavity, whereas the parotid gland is the primary site in three fourths of major salivary gland SCCs. One half of major salivary gland and three fourths of paranasal sinus SCCs have only local disease at presentation. Both patients in this report developed bone marrow metastases, a feature heretofore not observed in SCC from these primary sites. The patient with maxillary sinus SCC developed the syndrome of inappropriate antidiuretic hormone (SIADH). CONCLUSION: The paranasal sinus and major salivary glands are rare primary sites for SCCs. Long-term survival with local therapy in patients with local disease can occur, but in patients with metastatic disease survival mirrors metastatic pulmonary SCC.

Bone Marrow↗