Osteomalacia cured by resection of cutaneous neurinoma

INTRODUCTION: Regression of osteomalacia after exeresis of a skin tumor is unusual. CASE REPORT: A 62-year-old man had suffered from bone and joint symptoms for several years due to osteomalacia which was confirmed both biologically and histologically. The patient also had a plantar neurinoma. After exeresis of the tumor the biological results returned to normal levels within one week followed by regression of the clinical signs of osteomalacia. DISCUSSION: The neurinoma in this patient was apparently the cause of osteomalacia, since signs of the disease disappeared after exeresis. To date, three cases of neurinoma associated with osteomalacia have been published, including a single case with skin localization. The tumor would secrete a substance which inhibits the synthesis of vitamin D and enhances phosphorus excretion.     

Pain in the family

A case is reported of atypical glomus tumor occurring in the posterior inferior mediastinum of a 26-year-old woman complaining of severe back pain. The tumor was composed of atypical small, round tumor cells with scattered mitotic figures. In addition to sheet-like, diffuse proliferation of the tumor cells, some areas of the tumor contained small "glomoid" cells arranged in organoid and hemangiopericytomalike patterns. Immunohistochemically, many tumor cells were positive for muscle-type actins and a few cells were focally positive for desmin. Ultrastructural studies revealed smooth muscle features of tumor cells, that is, pinocytotic vesicles, external laminas, dense plaques, and occasional thin filaments with dense bodies. The patient remained well for 5 years and 4 months after the operation without additional radiation and chemotherapy. The tumor was diagnosed as an atypical, or low-grade malignant, glomus tumor morphologically. It seems important to recognize the presence of this type of tumor in sites other than extremities and to differentiate it from other malignant small, round cell tumors.     

Metastasis to the small intestine of malignant melanoma as a rare cause of intestinal hemorrhage

This is the case report of a 63 year old female patient, who was admitted to the hospital due to an unexplained anemia. A malignant melanoma of the cheek was excised four years previously (stage II, Clark level V, TNM classification: pT4, pNl, MO), followed by chemotherapy. By x-ray and CT examination an intestinal malignant growth was assumed. Laparotomy revealed a metastasis of the malignant melanoma, situated in the middle of the jejunum as cause of the occult bleeding. Complete resection of the tumor was successfully carried out. A second look laparotomy one year later revealed no further tumor growth in the abdominal cavity.     

Neural variant of fetal rhabdomyoma and naevoid basal cell carcinoma syndrome

A case of intramedullary melanocytoma of the medulla oblongata; which has not been hitherto reported, is presented. The tumor ran on a slow progressive course. The patient died of pneumonia seven months after the operation. MRI findings did not specify this tumor as they were not distinct from those of malignant melanoma. The tumor was totally black and was not attached to the dura. Microscopically, the neoplastic cells were uniform in size and possessed abundant melanin granules in their cytoplasm. Mitosis was very rare. The ultrastructure of the tumor cells showed many melanosomes at varying stages of maturation. The melanocytoma in the eloquent region has an unfavorable outcome.     

Synchronous glottic granular cell tumor and subglottic spindle cell carcinoma

We report the clinicopathologic findings in a case of coexistent glottic granular cell tumor and subglottic spindle cell carcinoma. There is no evidence in this case that suggests malignant transition of the granular cell tumor to malignant tumor. To our knowledge, this is the first report of synchronous granular cell tumor and subglottic spindle cell carcinoma.     

The use of oral opioids in patients with chronic non-cancer pain. Management strategies

Recurrence is a major cause of death in patients with medulloblastoma. Although the exact protocol for regular radiographic evaluation is sometimes a matter of debate, continuous follow up is necessary. We report a case of long-term survival after surgical removal of a subfrontal recurrent medulloblastoma, which occurred more than 3 years after total gross excision of the primary lesion and radiation therapy. The asymptomatic recurrence, which was detected by routine follow-up neuroimaging tests, was excised. The patient subsequently received gamma knife irradiation of the tumor bed followed by a course of "8 in 1" chemotherapy. "Early delayed radionecrosis" occurred 13 months after gamma knife treatment, which resolved spontaneously. Particularities of the case are discussed with reference to the location of the recurrent tumor, the possible pathogenetic causes and the side effects of treatment. We believe that this case and others reported in the literature underline the importance of continuous MRI surveillance of patients operated on for medulloblastoma.     

Age-related changes of electrocardiographic wave

OBJECTIVE: Cystic tumors of the adrenal gland are uncommon, but are being increasingly more frequently diagnosed during routine radiological evaluation as "incidentalomas". We discuss the differential diagnosis, therapeutic approach and the existing controversies concerning the management of this tumor type. METHODS: Two additional cases of adrenal pseudocyst in two women aged 47 and 38 years are presented. In one case the tumor was discovered incidentally, whereas the other case presented with acute pain arising from intracystic hemorrhage. RESULTS: The fist patient had a cystic tumor of 8 cm with some inner walls. Fine needle aspiration biopsy revealed a benign cystic lesion of the right adrenal gland. At laparotomy, an 8.5 x 4.5 cm multiloculated cystic lesion was excised. The second patient presented with abdominal pain due to intracystic hemorrhage. A Doppler US did not disclose any vessel inside the lesion. We performed a lumbotomy and excised a 7.5 x 6 cm cystic tumor located in the right adrenal gland. Both lesions were diagnosed as adrenal pseudocyst; the second case was a hemorrhagic one. CONCLUSIONS: The therapeutic approach in adrenal cystic tumors can be based upon the radiological and cytological findings since malignant cystic tumors are uncommon. A clear liquid and a negative cytology practically discard malignant tumors. Furthermore, cystic adenocarcinomas are usually large and the cystic liquid is cloudy with abundant cellularity. Surgical treatment is justified in the symptomatic, big or complex tumors (mixed, non-homogeneous).     

Primary bilateral malignant melanoma of the choroid (author's transl)

Report on an extremely rare case of primary bilateral malignant melanoma of the choroid with histological verification of both tumors. The patient was a 54-year-old woman; the presumptive clinical diagnosis was a primary bilateral melanoblastoma. A thorough general examination did not reveal any metastases. Six months later the patient underwent surgery. The left eye, in which the tumor was larger, was enucleated and a B-type malignant melanoma of the choroid with invasion of the sclera was diagnosed histologically. Two months later the patient died of a lung embolism. Histological verification of the tumor of the right eye led to the same diagnosis as in the left eye, but without invasion of neighbouring structures. No distant metastases were found at autopsy. A thin layer of slender spindle nevus cells was found between the tumor and the sclera during the histological study. The author agrees with Yanoff and Zimmerman (1967) that this may be regarded as the origin of the malignant tumor.     

Diagnosis and therapy of malignant lymphedema

In addition to the typical clinical symptoms, the diagnosis "malignant lymphedema" also requires the confirmation of a tumor or a metastasis obstructing lymph flow. With the standard physical treatment of edema described by Asdonk successful clinical management of malignant lymphedema is also possible. The sole contraindication of manual lymph drainage is, we believe, locoregional tumor recurrence, which can be completely eliminated by the immediate initiation of radical tumor treatment. The tumor recurrence is the result not of falsely indicated manual lymph drainage, but of inadequate primary treatment that leaves behind residual tumor tissue, the early detection of which still remains an unresolved problem. Manual lymph drainage is indispensable for improving the quality of life of tumor patients with lymphedema.     

Stability study of epirubicin in NaCl 0.9% injection

We report a rare malignant endovascular papillary angioendothelioma (Dabska tumor) arising within the deep periosteal soft tissue of the ulna of an 8-year-old girl. To our knowledge, this is the first report of a Dabska tumor of deep soft tissue that did not extend down from the dermis. This Dabska tumor appears to be a focal change within a larger, well-defined, cavernous hemangioma of deep muscle. We suggest that this represents secondary neoplastic progression of an existing benign lesion, a common phenomenon among vascular tumors. A review of the family of "hobnailed" endothelial cell lesions to which Dabska tumor belongs is presented.     

Capsule-bending ring for the prevention of capsular opacification: a preliminary report

An association between mediastinal germ cell tumors (MGCT) and hematological malignancies (e.g. acute leukemia, malignant histiocytosis) has been recognized since 1984. A rare case of mediastinal mature teratoma with angiosarcoma, a hematopoietic region and granulocytic sarcoma is reported in a 29-year-old male. The resected tumor was 9.0 x 6.5 cm, weighed 65 g and showed extensive necrosis, forming a cyst. The histological features of the tumor showed a mature teratoma, which contained a large gland lined by ciliated epithelium, hyalinous cartilage, a paraganglion-like structure, well-differentiated angiosarcoma with atypical hematopoiesis composed of CD34-positive cells, and malignant round cells. The malignant round cells did not stain for CD34 but were positive for leukocyte common antigen (LCA) and c-kit product. From these findings, the round cells were diagnosed as granulocytic sarcoma. The patient died of metastasis of the granulocytic sarcoma in the tonsils and cervical lymph nodes 8 months after surgery. A leukemic condition was not present throughout the clinical course. The association between MGCT and hematological malignancy is a distinctive syndrome. However, its pathogenesis is still obscure and the origin of the hematopoietic malignancy has not been fully elucidated. In this particular case, it is suggested that the granulocytic sarcoma might have arisen from the abnormal hematopoietic area in the mediastinal teratoma.     

Severe erosive stomatitis: association with immunological diseases?

This report presents a 63-year-old Caucasian woman with a malignant blue nevus, which is an extremely rare form of melanoma originating from or associated with a preexisting blue nevus. The background blue nevus on the left upper arm, which had been present for 5 to 6 years, increased in size and darkened in color for 3 months prior to histological diagnosis of malignant blue nevus. Although the tumor looked much like a nodular melanoma clinically, the diagnosis of malignant blue nevus was established histologically. The patient had a poor outcome due to metastatic spread of the tumor to the visceral organs 1 year following the initial excision of the tumor. To distinguish this rare tumor from other melanocytic lesions, strict histological criteria are needed to make the diagnosis of malignant blue nevus. Differential diagnosis includes cellular blue nevus, atypical cellular blue nevus, primary malignant melanoma, and metastatic melanoma to the dermis. Malignant blue nevus is most commonly seen on the scalp. The tumor has an aggressive behavior and metastasizes in the majority of patients. This paper describes the second reported case of malignant blue nevus involving the upper arm. Clinical and histological features of this uncommon tumor are presented, along with a review of the literature.     

Hypercalcaemia in patients with newly diagnosed tuberculosis in Malaysia

A giant hidradenocarcinoma presented by a 75-year-old female is reported. The patient had a malignant transformation within a nodular hidradenoma involving the right postauricular area, which was treated by mass removal and a right radical neck dissection with a free-flap covering. Malignant hidradenocarcinoma is the least common adnexal tumor of uncertain origin. They are usually malignant from their inception, but some develop from a benign counterpart. To the authors' knowledge, only three cases have been reported previously. Two histologically distinct components were seen in this tumor: (i) typical nodular hidradenoma, which constituted a small part of the tumor; and (ii) carcinoma with areas of transition. The secretory cells of hidradenocarcinoma showed decapitation secretion on light and electron microscopic observations, which is evidence of apocrine differentiation. Histologically, this case was concluded as a hidradenocarcinoma arising from a long-standing nodular hidradenoma. A literature review is presented and the histological, immunohistochemical and ultrastructural features are described.     

Gliofibromas (including malignant forms), and gliosarcomas: a comparative study and review of the literature

The presence of connective tissue elements in gliomas necessitates in every case a thorough analysis of the character and derivation of such elements to allow the formulation of an appropriate diagnosis. Four cases are presented in this paper. In cases 1 and 2 (anaplastic astrocytomas in two children, 9 and 4 years old, respectively) all the neoplastic elements were astrocytes and their ability to produce or indirectly promote the production of reticulin and collagen fibers accounted for the presence of such elements in close association with the tumor cells. The term "gliofibroma" has been coined for such tumors, but "desmoplastic astrocytoma", (low grade or anaplastic) or in highly malignant cases "desmoplastic glioblastoma", as the case may be, also seem to be appropriate terms for such neoplasms. In contrast, cases 3 and 4 represented composite tumors in adults (66 and 58 years old, respectively) and the neoplasms of these patients consisted of glioblastoma and sarcoma, the latter component demonstrably being of vascular origin. This is the type of tumor usually referred to as gliosarcoma or "Feigin tumor". Although some apparent similarities between the two groups may exist at times, the histogenesis of the latter group's sarcomatous or sarcoma-like portions is different from that of the first group and, therefore, warrants separate diagnostic terms and placement in brain tumor classification.     

Percutaneous treatment of bronchial artery aneurysm with use of transcatheter coil embolization and thoracic aortic stent-graft placement

Lactic acidosis due to thiamine deficiency is known to complicate chemotherapy and radiotherapy treatment of malignant extracranial tumors, but to the authors' knowledge, this complication has not been reported in patients treated for malignant brain tumors. They report three such cases, demonstrating that this complication can occur during treatment of brain tumors. In all patients, consciousness levels deteriorated within 1 to 2 days. Serum lactic acid levels increased to concentrations between 62 and 96.7 mg/dl, resulting in severe metabolic acidosis. A low blood thiamine level (9 ng/ml) was demonstrated at the onset in one case, and high-dose thiamine infusions dramatically improved lactic acidemia as well as impairment of consciousness in two cases. In the other case, hydrocephalus was suspected initially, resulting in a delay in thiamine supplementation. Clinical differentiation of this form of lactic acidosis from hydrocephalus or tumor progression can be very difficult in a patient undergoing treatment for a malignant brain tumor. Demand for thiamine is thought to be increased in patients with malignant brain tumors, and supplemental thiamine during treatment is necessary to prevent lactic acidosis. When this complication occurs, immediate treatment with sufficient thiamine is essential, together with normalization of pH by using sodium bicarbonate. With timely intervention, the level of consciousness can recover to the preacidotic state with no new neurological deficits.     

Umbilical metastasis of an endometrial adenocarcinoma: "Sister (Mary) Joseph's nodule". Review of the literature

In the beginning of the XXth century, Sir Hamilton Bailey proposed the name "Sister Joseph's nodule" for the umbilical metastasis of an abdominal cancer. This unusual pathology has multiple primitive etiologies. We report here the 27th case of an umbilical metastasis of an endometrial adenocarcinoma. In case of a malignant umbilical tumor, 75% correspond to a "Sister Joseph's nodule". Their clinical manifestations are quite similar. This secondary localisation could appear before, during or after the diagnosis of the primitive tumor. Adenocarcinoma is the frequently diagnosed histological type. The endometrial origin represents only 1.4% of the cases. Multiple routes of spread exist. Prognosis remains poor. Medico-surgical treatment, in a curative target, will be aggressive and should be adapted at every case. Our case-report recalls a new patho-physiological approach. The extended follow-up of this patient, without further medical treatment, is an additional argument.     

Fine needle aspiration cytology of malignant chondroid syringoma: a case report

BACKGROUND: Chondroid syringoma, a tumor of the eccrine glands, was previously called mixed tumor of skin as it has both mesenchymal and epithelial elements. Malignancy in this tumor is extremely rare. Although there are a few reports describing the cytomorphologic features of chondroid syringoma, the cytologic findings of its malignant counterpart have not been described. CASE: A 40-year-old female presented with a recurrent swelling on the scalp of one year's duration. Fine needle aspiration yielded blood-mixed gelatinous material. May-Grünwald-Giemsastained smears showed epithelial cells arranged in cordlike structures and ill-formed glands against a myxomatous background. The epithelial cells had scanty cytoplasm and markedly pleomorphic nuclei with prominent nucleoli. A few cells in the stroma had a halo around them and a resemblance to cartilage cells. A preoperative diagnosis of malignant chondroid syringoma was made. The tumor was excised, and the cytologic diagnosis was confirmed on histopathology. CONCLUSION: Cytomorphologic features of a rare case of malignant chondroid syringoma are reported for the first time. The presence of malignant epithelial cells against a myxoid background with a few chondroid foci helped in making a correct preoperative diagnosis.     

Malignant carcinoid tumor and synchronous malignant extraadrenal paraganglioloma

Gastrointestinal carcinoid tumor is often considered the most common neuroendocrine tumor of the small intestine. The overall incidence of 1% in the general population is quite low. Extraadrenal paragangliomas are rarer still, and the incidence of both of these tumors in the malignant state is exceedingly rare. This article describes the case of a patient who had both a malignant carcinoid tumor as well as a malignant retroperitoneal paraganglioma occurring synchronously. A review of the literature concerning these tumors is also presented.     

Plasma platelet-activating factor levels in newborn infants with and without perinatal asphyxia: is it an additional marker of perinatal asphyxia?

We report a case of severe hyperthermia in a 6-month-old boy with a single ventricle, dextrocardia, asplenia, and transposition of the great arteries, during induction of anesthesia on three separate occasions. To our knowledge, this is the first case reported of repeated intraoperative hyperthermia not related to malignant hyperthermia, infection, neuroendocrine tumor, or iatrogenic causes (e.g., anticholinergic blockade or warming devices). The severe hyperthermia may be secondary to the medications given before and during induction and/or the stress of the induction. Among the induction medications given during the three episodes, fentanyl is the most likely contributing drug. Human data indicate that opioids increase the sweating threshold and decrease the vasoconstriction and shivering thresholds. The medications could cause a widening in the thermoregulation interthreshold and the stress could induce nonshivering thermogenesis.     

Anaplastic pleomorphic xanthoastrocytoma

Well-documented cases of malignant degeneration in pleomorphic xanthoastrocytoma and of anaplastic pleomorphic xanthoastrocytoma are rare in the literature. We report 2 cases of pleomorphic xanthoastrocytoma, 1 of which demonstrated clear evidence of malignant degeneration in the absence of prior radiation therapy over an 18-year period. Both anaplastic tumors were characterized by foci of necrosis and increased mitotic activity (3 and 2 mitotic figures/10 high-power fields). Both tumors demonstrated focal positive staining for glial fibrillary acidic protein and showed marked reticulin deposition. An MIB-1 labeling index (marker of cell proliferation) in the initial low-grade-appearing tumor in case 1 was 0.1%. The recurrent tumor in case 1 had an MIB-1 labeling index of 4.9%, and the anaplastic tumor in case 2 had an MIB-1 labeling index of 5.4%. Significant cyclin D1 immunoreactivity was not observed in either anaplastic tumor. Two percent to 3% of tumor cells stained positive with p53 protein antibody in the recurrent anaplastic tumor in case 1. Although histology may not reliably predict aggressive behavior in pleomorphic xanthoastrocytomas, the presence of increased mitosis, necrosis, and increased cell proliferation labeling indices may be indicative of a higher grade tumor.