Malignant triton tumor of the kidney in a child: A case report

IntroductionMalignant triton tumor (MTT) is an extremely rare variant of the malignant peripheral nerve sheath tumors (MPNSTs) with rhabdomyosarcomatous differentiation, which was first described in 1932 by Mason. MTT affects, in most cases, patients under 35 years of age, and it is usually manifested as a mass that may or not be painful. However, the incidence in pediatric patients is atypical. This tumor presents an aggressive course and limited survival rate, and the prognosis is different between individuals with or without a concomitant diagnosis of neurofibromatosis type 1 (NF1). Currently, the recommended treatment is surgical resection, and adjuvant chemotherapy and radiotherapy, but its efficacy is not yet clear.Presentation of the caseA 13-year-old female patient was referred to the pediatric oncology service due to the presence of an abdominal mass and weight loss, initially diagnosed with Wilms' tumor. After extensive investigation, surgical resection, and immunohistopathological evaluation, the diagnosis of malignant triton tumor was confirmed. The patient also underwent cycles of chemotherapy after resection, and is currently awaiting immunotherapy.Discussion and conclusionMalignant triton tumor is extremely rare and difficult to diagnose, especially in children or young people, age groups in which the incidence of the disease is even lower. This may be the reason it is rarely suspected, and it was a great challenge for the clinical care team. It is essential to consider and investigate this possibility of differential diagnosis, as patients diagnosed with this malignant tumor have a low survival rate and poor prognosis.     

Malignant solitary tumor in a child: a case report and review of the literature

Solitary fibrous tumor in the mesentery is rare. We report a case of a malignant solitary fibrous tumor in the appendical mesentery of 6-year-old boy. Computed tomography and ultrasound of the abdomen demonstrated a well-defined solid mass, 4.0 cm in diameter, in the lower right abdomen. At laparotomy, an encapsulated tumor was observed in the appendical mesentery and was easily separated from the appendix. Immunohistochemistry stain showed that the spindle-shaped cells were positive for CD34 and neuron-specific enolase. DOG-1, CD117, desmin, S-100 protein, and SMA were negative. The sequence of KIT and platelet-derived growth factor receptor α was presented with wild type, no mutation. The patient received the reoperation 5 months postoperation. Despite these treatments, the relapsed tumor rapidly and markedly enlarged. The patient died of recurrent tumor with liver dissemination 7 months after reoperation.     

前列腺恶性叶状肿瘤(附一例报告并文献复习)

目的　探讨前列腺恶性叶状肿瘤的临床、病理特征及治疗方法。　方法　分析 2 0 0 3年1月收治的 1例前列腺恶性叶状肿瘤患者的临床资料 ,并进行文献复习。患者 6 0岁 ,因排尿困难伴间歇性肉眼血尿 1年 ,TURP术后症状复发 2个月入院。直肠指诊示前列腺 5cm× 6cm ,质软 ,表面光滑。血清PSA 1.2 5ng/ml,CT检查示前列腺密度不均。复阅病理切片后诊断为前列腺肉瘤 ,全麻下行根治性前列腺切除术。　结果　术后病理检查肿瘤类似乳腺叶状囊肉瘤 ,由上皮和间质成分构成 ,其中间质细胞明显增多 ,细胞有异型性 ,可见核分裂相。上皮细胞增生 ,但无明显异型性。由上皮细胞形成的囊腔被增多的间质成分挤压、拉长形成类似叶状的裂隙。精囊及膀胱颈部均有肿瘤细胞浸润 ,诊断前列腺恶性叶状肿瘤 ,标本切缘阴性。免疫组化染色检查 :间质细胞Vimentin( ) ,Actin( - ) ,上皮细胞CK( ) ,PSA( ) ,AE1/AE3( ) ,基底细胞CK34BE12 ( )。患者于术后 6个月肿瘤复发 ,拒绝进一步治疗 ,目前仍在随访中。　结论　前列腺恶性叶状肿瘤罕见 ,确诊依靠其特异性临床及病理表现 ,根治性前列腺切除术是目前最可靠的治疗方法     

Malignant glomus tumor of the foot. Case report

Introduction and importanceGlomangiosarcoma or is an extremely rare tumor. Few cases are published in the literature.Case presentationWe present a rare case of 64-year-old female patient presented small reddish subcutaneous nodules on the sole of the foot. Surgical resection revealed malignant glomus, the evolution was marked by an infiltrating local recurrence leading to amputation, without notable metastases after six months.Clinical discussionMalignant glomus tumor exhibit unusual characteristics, notably deep localization, large size and infiltration, mitotic activity, nuclear pleomorphism and mitonecrosis. The wide excision and possible amputation for infiltrating local type unresecable was the adequate treatment.ConclusionGlomangiosarcoma arinsing de novo are exceedingly rare and must be considered the most aggressive and with high potential risk of metastasis.     

Malignant pancreatic endocrine tumor in a child with tuberous sclerosis

Tuberous sclerosis complex (TSC) is an autosomal dominant condition whose signs and symptoms may vary from a few hypopigmented skin spots to epilepsy, severe mental retardation, and renal failure. The disease is caused by mutations in either TSC1 or TSC2 gene, at chromosome 9q34 and 16p13.3. Inactivation of both alleles at TSC1 or TSC2 loci is associated with the development of hamartomas in different organs, and only rarely with malignant neoplasms. In this study we present a 6-year-old boy with TSC and with a malignant islet cell tumor of the pancreas. Mutation analysis of DNA extracted from peripheral blood cells of the patient identified an R1459X de novo mutation in exon 33 of the TSC2 gene. Immunohistochemical analysis with anti-tuberin antibodies on paraffin-embedded tissue sections showed loss of tuberin immunostaining in tumor cells but normal expression in residual normal pancreas. DNA analysis of tumor and normal cells showed chromosome 16p13 loss of heterozygosity in malignant pancreatic islet cell tumor but not in normal pancreas. These findings suggest a role for tuberin, the TSC2 gene product, in the pathogenesis of malignant pancreatic endocrine tumor.     

Malignant melanoma in a child. A case report

The rare occurrence of malignant melanoma in a child is reported. Differentiation on histopathological grounds between this tumour and the more common juvenile melanoma may be difficult, but this important distinction should be possible in almost all cases.     

Bednar tumor of the foot: a case report

Pigmented dermatofibrosarcoma protuberans (Bednar tumor) is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans. The majority occurs on the trunk, and the remainders are more or less equally distributed in the upper and lower extremities and the head and neck. Microscopically it is characterized by spindled cells arranged in a tight storiform pattern and admixed with a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature that distinguish this lesion from conventional dermatofibrosarcoma protuberans. We report here a case of Bednar tumor occurring on the dorsal aspect of the foot in a young female.     

乳腺恶性纤维性组织细胞瘤1例

患者　女 ,40岁。因左乳包块2个月入院。曾于 7个月前行左乳肿物的切除术 ,未送病理。体查 :左乳晕外上侧可见 1弧形切口疤痕长 4ｃｍ ,其下方扪及 4ｃｍ× 4ｃｍ× 3ｃｍ肿物 ,质硬 ,活动度差 ,与皮肤有粘连。诊断 :左乳癌。在全麻下行乳癌根治术。镜下瘤组织有多种细胞成分 ,纤维母细胞、组织细胞及多核巨细胞等。纤维母细胞呈梭形 ,具一定异型性 ,形成胶原纤维束 ,作车辐状排列。Ｖｉｍｉｔｔｉｎ ( )、ＥＭＡ(- )。病理报告 :左乳腺恶性纤维组织细胞瘤。讨论　乳腺肉瘤少见 ,只占乳腺恶性肿瘤的 2 %。肉瘤中间质肉瘤又远比恶性叶状囊肉…     

Malignant hyperthermia in a black child. A case report

A case of malignant hyperthermia in a Black boy is presented. He developed this condition during repair of a cleft palate, with halothane as the triggering agent. The importance of the high incidence of malignant hyperthermia in patients with certain musculoskeletal abnormalities is stressed. Despite a cool and well air-conditioned theatre, the patient's temperature was 41 degree C when the condition was suspected. At that stage general muscle rigidity was present. The patient was successfully treated with procainamide, sodium bicarbonate and hydrocortisone; surface cooling (with ice packs) was instituted and the stomach was washed out with ice-cold Ringer's solution. Over a period of 14 days serum creatine phosphokinase values decreased from 630 IU (on the day of the incident) to 12 IU. A muscle biopsy showed variation in muscle fibre size. Electron microscopical studies showed myofibrillar disruption and folding of the basement membrane. A modified version of Denborough's technique was used for the in vitro exposure of muscle strips to halothane and suxamethonium. Isometric contraction was measured and recorded. A severe contraction followed the exposure of muscle strips to halothane, which confirmed the diagnosis.     

Malignant carotid body tumor: a case report

Carotid body tumors (CBTs) have an unpredictable history with no correlation between histology and clinical behavior. Of reported cases since 1891, local and distant metastases appear in approximately 10% of cases and remain the hallmark of malignancy. Currently, there are not enough data to support a single treatment regimen for malignant CBTs. The reported case demonstrates some unanswered issues with regard to malignant CBTs to include lymph node dissection, the need for carotid resection, and the role of radiation therapy. A 46-year-old pathologist underwent a resection of a Shamblin I CBT, to include jugular lymph node sampling, without complication. There was lymph node involvement, and tumor cells were found on the margins of the pathologic specimen. Subsequent carotid resection with reversed interposition saphenous vein graft and modified neck dissection were performed again without complication. Follow-up at 4 years has been uneventful. Diagnosis of CBTs with the use of magnetic resonance angiography, magnetic resonance imaging, color flow duplex scanning, and the role of arteriography are reviewed. The current treatment options are discussed with reference to primary lymph node sampling, carotid resection, and neck dissection in malignant cases. This case demonstrates that the unpredictable nature of CBTs and their malignant potential warrant aggressive initial local treatment to include jugular lymph node sampling and complete tumor resection.     

Malignant schwannoma of the digital nerve in a child. A case report

An extremely rare case of malignant schwannoma (malignant peripheral nerve sheath tumor) arising in the digital nerve in the right ring finger of a child is described. The patient was an 8-year-old boy who did not have neurofibromatosis (von Recklinghausen's disease). He noticed a small tender mass on the tip of his right ring finger in 1991. The lesion was excised twice. However, the tumor recurred soon after each surgery. Histologic examination revealed malignant schwannoma. He eventually underwent disarticulation of the ring finger at the metacarpophalangeal joint in 1995. There was no recurrence or distant metastasis at followup in July 1999. Solitary malignant schwannomas can occur in the small peripheral nerves and in the major nerves.     

Management of foot pain associated with accessory bones of the foot: two clinical case reports

STUDY DESIGN: Case study. OBJECTIVES: To discuss the differential diagnosis, the nonsurgical and postoperative management of common accessory bones of the foot. BACKGROUND: Accessory bones of the foot that are formed during abnormal ossification are commonly found in asymptomatic feet. Two of the most common accessory bones are the accessory navicular and the os peroneum. Their painful presence must be considered in the differential diagnosis of any acute or chronic foot pain. The optimal treatment for the conservative and postoperative management of painful os peroneum and accessory navicular bones remains undefined. METHODS AND MEASURES: Therapeutic management of the fractured os peroneum included bracing, taping, and foot orthotics to allow healing of involved tissues, and stretching. The focus of the postoperative management of the accessory navicular was joint mobilization and progressive strengthening. Dependent variables included level of pain with provocation and alleviation tests of joint and soft tissue; girth and sensory tests of the foot and ankle; goniometric measures of foot and ankle; strength of ankle and hip muscles; functional tests; and patient's self-reported pain status. RESULTS: The patient with the fractured os peroneum was treated in 13 visits for 10 weeks. At discharge from physical therapy, the patient had the following outcomes relative to the noninvolved side: 100% return of normal sensation tested by light touch and vibration; pain decreased from 6/10 to 1/10; 100% reduction of swelling with ankle girth to normal; 100% range of motion of ankle and subtalar joints. Strength in plantar flexion and eversion remained 20% impaired (80% return to normal) secondary to pain. Upon discharge, he still reported mild pain when walking but was able to return to previous leisure activities. The second patient with the accessory navicular was treated in 18 visits over 9 weeks. Relative to the uninvolved side, she was discharged with the following: 70% return of range of motion in the foot and ankle, 100% of strength in hip and ankle, and 100% return of balance. She could squat and jump without pain and she returned to full premorbid activity level. CONCLUSIONS: Rehabilitative management of both cases addressed specific impairments and was successful in improving the patients' activity limitation. Clinicians should be aware that these accessory bones are possible sources of disability, secondary to foot pain.