Colonic neuroendocrine carcinoma in a child

A 10-year-old boy with congenital immunodeficiency (X-linked agammaglobulinaemia) presented with loss of appetite and weight, right-sided abdominal pain, diarrhoea and low-grade fever. Radiological investigations with barium follow-through, CT, PET and octreotide scans revealed a primary caecal/ascending proximal colonic mass with liver and bony metastases. Urine screen for 5HIAA was positive. Percutaneous liver biopsy confirmed the diagnosis of neuroendocrine carcinoma. The radiological work-up and the usefulness of various imaging modalities in the diagnosis of this rare paediatric tumour are discussed. The PET scan demonstrated the primary tumour and the metastatic locations more vividly than the octreotide scan, which is currently considered to be the most specific imaging modality for neuroendocrine masses.     

Fetus in fetu: two case reports and review of literature

Fetus in fetu is a rare disorder. Its embryopathogensis and differentiation from teratoma has not been well established. It is a parasitic twin of a diamniotic monozygotic twin. Here we report, two cases of fetus in fetu with review of literature. In case report 1, a 2-year-old boy was referred for asymptomatic lump in abdomen since birth. X-ray showed the mass in the abdomen with some calcification and fluid inside. CT scan reported a heterogenous mass in the retroperitoneum with bony malformation. CT showed presence of three vertebrae in it. After surgically excising the mass and opening the sac it showed presence of trunk and two limbs with one of the limbs having a nail. Histopathology showed presence of GI tract. In case report 2, 4 month female was found to have lump in the abdomen by housemaid while bathing. X-ray showed a soft tissue shadow while ultrasonography revealed cystic mass arising from right kidney. CT suggested cystic mass with calcification not arising from kidney. During exploration whole mass was excised and there was frank fetus inside it. Histopathology confirmed presence of four vertebral bodies with germ layers. Although fetus in fetu is rare condition, correct diagnosis using imaging can be made before surgery. Complete excision is curative.     

Mature cystic teratoma of the pancreas in a child

A cystic pancreatic tumour is rare in a child and a mature cystic teratoma of the pancreas is even rarer. This is the first demonstration of the CT appearance of such a tumour in a child. We present a 2-year-old boy who presented with a palpable abdominal mass. Abdominal CT revealed a huge cystic mass in the upper abdomen. Pathology disclosed a mature cystic teratoma originating from the pancreas.     

Mucoepidermoid carcinoma of bronchus in a pediatric patient: <Superscript>18</Superscript>F-FDG PET findings

In children, primary neoplasms of the tracheobronchial tree and lungs are rare; most are malignant. Of the primary malignant pulmonary neoplasms arising in childhood, mucoepidermoid carcinoma accounts for approximately 10%. Due to its well-confined local growth within the airway, mucoepidermoid carcinoma commonly produces respiratory symptoms from progressive tracheal or bronchial obstruction. Mucoepidermoid tumor has minimal metastatic potential in children, and local resection alone is the current treatment of choice. Early detection, diagnosis, and surgical resection of mucoepidermoid tumor are especially important in pediatric patients since the bulk of the remaining pulmonary parenchyma can be preserved, thereby decreasing the thoracic deformity and pulmonary functional morbidity. Radiographic and CT imaging findings of bronchial mucoepidermoid carcinoma in children have been described in several case reports. However, to the best of our knowledge, imaging findings of 2-(¹⁸F)-fluoro-2-deoxy-d-glucose positron emission tomography (¹⁸F-FDG PET) of mucoepidermoid carcinoma of the bronchus in pediatric patients have not been well established. We report a mucoepidermoid carcinoma arising from the right upper lobe bronchus in a 15-year-old girl with an emphasis on the ¹⁸F-FDG PET findings.     

Characteristic imaging features of desmoplastic small round cell tumour

Background

Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described.

Objective

To assess the CT parameters characteristic of DSRCT. We also report our experience with combined FDG PET/CT in staging and follow–up for DSRCT.

Materials and methods

The pretreatment diagnostic CT’s of 65 patients with DSRCT were evaluated. Pertinent imaging findings were catalogued, with histopathology or serial follow-up studies as reference standard. Combined FDG PET/CT examinations of 11 of these patients who underwent pretreatment imaging were also reviewed.

Results

Sixty-two patients presented with primary intra-abdominal disease; three had primary extra-abdominal tumours at presentation. The most common imaging finding of patients with intra-abdominal DSRCT was multiple peritoneal soft tissue masses, with a dominant mass in the retrovesical or rectouterine location in more than half of the cases. Forty percent had metastatic disease to the liver, lungs, spleen or bones at diagnosis. FDG PET/CT accurately detected 97.4% of all DSRCT lesions.

Conclusion

DSRCT typically presents as a large abdominopelvic mass with widespread peritoneal involvement predominantly in young males. Familiarity with its radiological features can help guide diagnosis and treatment. Functional imaging with PET/CT offers advantage over anatomical imaging for accurate disease staging.     

Whole-body MR imaging with the use of parallel imaging for detection of skeletal metastases in pediatric patients with small-cell neoplasms: comparison with skeletal scintigraphy and FDG PET/CT

BACKGROUND: In pediatric patients with small-cell tumors, there is an increasing demand for accurate and early detection of skeletal, especially bone marrow, metastases as new treatment protocols are introduced. Whole-body MR imaging (WB-MR) and (18)F-fluorodeoxyglucose PET/CT (FDG PET/CT) are new promising imaging methods that can detect metastases before osteoblastic host response occurs, which is the basis for detection of metastases by skeletal scintigraphy (SSC). OBJECTIVE: To assess the ability of WB-MR to detect marrow metastases in children with small-cell neoplasms and compare its performance with that of FDG PET/CT and SSC. MATERIALS AND METHODS: During a 16-month period, 26 children and adolescents with histopathologically proven small-cell neoplasms underwent WB-MR, FDG PET/CT and Tc-phosphonate-based SSC in a random order within a 25-day period. Metastases were localized in relation to eight regions of the body. RESULTS: WB-MR revealed metastases in 39 out of a total of 208 regions in 26 patients (sensitivity 97.5%, specificity 99.4%, positive predictive value 97.5%, negative predictive value 99.4%), SSC in 12 regions (sensitivity 30%, specificity 99.4%, PPV 92.3%, NPV 85.6%) and FDG PET/CT in 36 regions (sensitivity 90.0%, specificity 100%, PPV 100%, NPV 97.7%). Both WB-MR and FDG PET/CT showed excellent agreement (kappa) with the final diagnosis (96.9% and 93.6% respectively), whereas SSC showed only moderate agreement (39.6%). CONCLUSION: Our results suggest that WB-MR and FDG PET/CT studies are robust imaging modalities for screening for skeletal metastases, and are far more accurate than SSC. The lack of radiation is an additional advantage of WB-MR, especially in the pediatric population.     

Extrarenal malignant rhabdoid tumor in childhood application of 18F-FDG PET/CT

Extrarenal malignant rhabdoid tumor (MRT) is a rare malignancy in childhood and has a poor prognosis. Accurate histopathologic diagnosis and staging of the malignancy has major implications for patient management. The application of F-fluoro-deoxy-glucose positron emission tomography/computed tomography (F-FDG PET/CT) in pediatric malignancy has been well described and is having a significant clinical impact in many common pediatric cancers, in particular lymphoma, brain tumors, bone and soft tissue sarcomas. The use of PET/CT using F-FDG in rare tumors such as MRT is unclear. Two cases of MRT in childhood are described. One patient, a 12-year-old female, was shown to have extensive metastatic disease on PET/CT, showed poor response to chemotherapy and progression of disease detected on PET/CT. Her management was changed to palliative care. The second child, a 20-month-old female, presented with a parapharyngeal mass. The initial magnetic resonance imaging showed the mass and possible metastatic ipsilateral cervical lymph nodes. The initial staging PET/CT confirmed avid metabolic activity in the tumor and regional node involvement but no distant metastases. She showed an initial good but incomplete response on PET/CT and magnetic resonance imaging to chemotherapy and her treatment program was changed. The patient relapsed with recurrent pharyngeal tumor and her management was changed to palliative care. MRT accumulate F-FDG avidly. PET/CT was helpful in the initial staging, assessing response to treatment, and in clinical decisions at various stages of management for both patients.     

Teratoma Associated with Endodermal Sinus Tumor

A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma.

The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.     

Mature teratoma arising from intra-abdominal contralateral undescended testis in an infant: a case report

A 4-month-old male infant was seen because of an asymptomatic undescended left testis and a right sided abdominal mass. CT revealed a calcified retroperitoneal tumour. Histological examination of surgical specimens showed a mature primary teratoma of the contralateral undescended testis. While this is very rare, infants with undescended testis should be carefully examined to rule out intra-abdominal malignant tumours.     

Paediatric gastric teratoma.

AIM: This paper reviews our experience with patients presenting with gastric teratoma treated over an eighteen-year period. This rare paediatric tumour has an excellent prognosis after curative resection. METHODS: There were seven patients, all males. Investigations included a haematological profile, plain radiography and ultrasound of the abdomen. All patients were operated and the specimens subjected to histopathological examination. RESULTS: The age at presentation ranged from 1 month to 7 years. Two patients presented with melaena and the rest with progressively increasing abdominal mass. Our oldest patient had a massive tumour occupying nearly the whole abdomen. None of the children had metastases and none of the lesions had invaded adjacent structures. The tumours could be completely resected and all patients had an uneventful recovery. Histology of the tumours showed mature elements arising from the three germ layers in 6 children, one child had an immature teratoma. The average follow-up was 3 years (range 2 - 6 years). None of the patients has had a recurrence or any other problem. CONCLUSIONS: Gastric teratoma is a rare tumour but amenable to curative resection with an excellent prognosis.     

An unusual mature thyroid teratoma on CT and 99Tcm scintigraphy imaging in a child

We report the imaging findings of a mature thyroid teratoma in a 5-year-old girl. Nuclear imaging showed a decrease in ⁹⁹Tcm uptake in the right lobe of the thyroid gland. CT scan showed a slightly lobulated soft-tissue mass without calcification, fat or cystic components. Histological analysis showed that the tumor was composed of mature neural tissue, cartilaginous, and epithelial elements. This case study provides new insights into the CT appearance of mature thyroid teratomas.     

Sacrococcygeal teratoma in Northeastern Nigeria: 18-years experience

Although sacrococcygeal teratomas are rare, there have been several reports from various parts of Nigeria, outside the Northeast region. This report reviews the experience with this tumour from the northeastern part of Nigeria. A retrospective study of 21 children with histologically confirmed sacrococcygeal teratoma managed in 18 years. Over the 18-year-period, 21 histologically confirmed cases of sacrococcygeal teratoma were seen—80% were girls. Thirteen (60.9%) presented during the neonatal period. Eighteen patients presented with benign sacrococcygeal teratoma while three patients and a recurrence from an initial benign tumour had malignant sacrococcygeal teratoma. All malignant tumours had a significant presacral component. There were associated congenital malformations in 3 (14.3%) of the patients. All the benign tumours were resectable. Using the American Academy of Pediatrics (Altman’s) classification (Altman et al., J Pediatr Surg, 9:389–398, 1974), 13 patients were type I, 5 patients type II, 3 patients type Ill and none was for type IV. The commonest post-operative complication was wound infection in six patients. Others were wound dehiscence in three and post-operative diarrhoea in two patients. Three deaths were recorded in this series. The size of mass greater than 5 cm did not correlate with the incidence of post-operative complications (P=0.367). The post-operative follow-up was very poor; but there was a recurrence in a girl, two-years after the initial surgery. Our study revealed that most of our patients with sacrococcygeal teratoma present early to hospital with benign lesions and the immediate post-operative results were excellent. However, the follow-up was poor as such long-term post-operative results are difficult to study. It is to be recommended that sacrococcygeal teratoma presenting early to hospital should have prompt surgical intervention.     

Whole-body MRI of paediatric malignant tumours: comparison with conventional oncological imaging methods

Purpose: To evaluate the usefulness of whole-body (WB) MRI for detecting metastases from paediatric malignant tumours in comparison with conventional oncological imaging methods. Materials and methods: Using a 1.5-T system, a coronal short tau inversion recovery (STIR) sequence was obtained in all patients. In addition, sagittal fat-suppressed T2-weighted, sagittal STIR, or coronal fat-suppressed pre-contrast and post-contrast T1-weighted sequences were performed. Patients who underwent WB MRI and conventional oncological imaging within 15 days were enrolled in the study. In total, 58 bone scintigraphies, 26 iodine-123 (¹²³I) meta-iodo-benzylguanidine (MIBG) scintigraphies, and 48 CT scans were available for comparison in 36 patients (median age 3.5 years; 21 boys, 15 girls) who underwent 82 WB MRI examinations. Skeletal and extraskeletal metastases were evaluated for a variety of tumour types. Results: Concordance rate of WB MRI between two readers was 74%. In detecting metastases, WB MRI had higher sensitivity (99%) and PPV (94%) than bone scintigraphy (26 and 76%, respectively). In detecting skeletal metastases, WB MRI revealed higher sensitivity (100%) than ¹²³I-MIBG scintigraphy (25%) and CT (10%). In contrast, WB MRI showed lower PPV in detecting skeletal and extraskeletal metastases (8 and 57%, respectively) than ¹²³I-MIBG scintigraphy (100%), and lower sensitivity (60%) in detecting extraskeletal metastases than CT (100%). In 2 of 11 untreated patients, tumour staging was upgraded from stage 3 to 4 according to WB MRI findings. In 3 patients, WB MRI revealed early treatment responses (<1 year) of skeletal metastases. Conclusions: WB MRI can substitute for bone scintigraphy in detecting skeletal metastases of paediatric malignant tumours, and it is useful in evaluating initial tumour staging and early treatment responses. However, it still has only a complementary role in detecting extraskeletal metastases.     

Teratoma of the stomach in a 4-day-old newborn infant

Teratoma of the stomach is a very rare tumor. Nearly all previously reported patients were males. We report a 4 day-old male infant, who presented with a palpable abdominal mass. A preliminary diagnosis of teratoma was made by abdominal x-ray, sonography and MR imaging. Surgery was performed and diagnosis of teratoma of the stomach was confirmed by histological examination of the tumor. Because teratoma of the stomach is always a benign neoplasm surgery is curative and no further therapy is needed.     

Mucinous cystadenoma of the ovary in perimenarchal girls

Ovarian masses in children are an uncommon occurrence. They represent less than 2% of all tumours in girls less than 16 years of age. Mucinous tumours of the ovary occur principally in middle adult life and are extremely rare prior to menarche. To the best of our knowledge, there are only 13 previous cases of benign mucinous cystadenoma (MCA) of the ovary in perimenarchal girls reported in the literature. We present six cases of this rare tumour. We reviewed the charts of six patients who presented with large MCA of the ovary. The patient’s ages ranged from 13 to 14 years (mean 13.6 years). Two were premenarchal and four were within 1 year of menarche. All children presented with marked abdominal distension and discomfort. Except for one child who had ultrasound scan alone, all the others had either CT or MRI scan as well. Ultrasound demonstrated a large multiloculated cystic mass arising from the pelvis reaching the level of the xiphoid. CT demonstrated an enormous mass occupying almost the entire abdomen. The mass was partly solid, partly cystic and the cystic elements were multiloculated in all patients. Three patients demonstrated contralateral hydronephrosis on imaging. Laparotomy revealed a tumour arising from the left ovary in five patients and from the right ovary in one. Several litres of fluid were aspirated in order to deliver the tumour from the abdomen. All patients underwent oophorectomy or salpingo-oophorectomy. Histology revealed benign MCA of the ovary in all cases. On follow up, ranging from 2.4 to 5 years, all patients were well with no evidence of recurrence. MCA in perimenarchal girls usually affects the left ovary. Although this tumour is rare, this diagnosis should be considered in 11 to 15-year-old girls presenting with a very large abdominal mass.     

Paradoxical response of a pineal immature teratoma to combination chemotherapy

A case of primary intracranial germ cell tumour is reported in a 5-year-old Chinese boy who presented with symptoms and signs of increased intracranial pressure. Computed tomography (CT) scan of the brain revealed a multicystic tumour over the pineal region. Stereotactic biopsy yielded enteric epithelium and isolated cells with hyperchromatic nuclei and high nuclear-to-cytoplasmic ratio, suggesting the diagnosis of an immature teratoma. Grossly elevated serum and cerebrospinal fluid (CSF) levels of α-fetoprotein and mildly elevated levels of β human chorionic gonadotrophin suggested the simultaneous presence of embryonal tissues. Combination chemotherapy with cisplatin, etoposide, and bleomycin resulted in a gradual but complete normalisation of the serum and CSF tumour markers. Paradoxically, the patient's consciousness deteriorated and repeat CT scan after 3 months of treatment showed that the tumour was actually increasing in size. Complete surgical removal was attempted and histologic sections of the specimen revealed only mature teratomatous tissues. The child is now well and remains disease-free with minimal left hemiparesis 12 months after completion of treatment. This case illustrates the importance of combined neoadjuvant chemotherapy and surgery in the management of intracranial non-germinomatous germ cell tumour harbouring both germ cell and teratomatous components, while monitoring of both biochemical and radiological parameters are necessary in assessing the clinical behaviour of mixed germ cell tumours. © 1995 Wiley-Liss, Inc.     

Colon carcinoma treated with oxaliplatin and capecitabine in a 12-year-old child

Background

XELOX (oxaliplatin 130mg/m² iv, capecitabine 1000mg/m² bid oral d1–14, q3w) chemotherapy has never been used in children. In this report, we present a case of a 12-year-old girl with colon adenocarcinoma, treated with surgery and XELOX chemotherapy.

Methods

On admission, the girl complained of abdominal pain and intestinal obstruction. Physical examination revealed a distended abdomen with tenderness on the left upper quadrant. Barium enema revealed a stenotic lesion at the distal end of the transverse colon, and abdominal computed tomography showed acute obstruction and a colonic mass. Laparotomy was performed after the failure of conservative treatment.

Results

The mass was originated from the transverse colon. Frozen sections of the specimens revealed an adenocarcinoma. Transverse colectomy was performed and regional lymph nodes were removed. Pathological examination confirmed that the mass was a poorly differentiated adenocarcinoma, and XELOX chemotherapy was used. No evidence of recurrent or metastatic tumor was found after 18 months.

Conclusion

Although complete resection is the most effective treatment, XELOX chemotherapy is beneficial to the improvement of clinical outcome of patients with colon adenocarcinoma.     

Primary intrathoracic lymphangioma masquerading as teratoma

Two cases of cystic hygroma arising as a primary lesion in the chest are presented. Because of calcifications discovered on CT and because of the anterior location of the mass in both cases, teratoma was the first diagnostic consideration. However, after surgical removal, histologic study determined that both lesions were cystic hygromas with areas of scattered calcification probably secondary to hemorrhage. These cases are presented because of the remarkable similarity of their imaging features to those of teratoma.     

Accuracy of 18F fluorodeoxyglucose positron emission tomography/computed tomography in staging of pediatric sarcomas

The present study was conducted to clarify the diagnostic accuracy of 18F-fluoro-2-deoxy-D-glucose (18FDG) positron emission tomography (PET)/computed tomography (CT) in the staging in pediatric sarcomas. Fifty pediatric patients with histologically proven sarcomas who underwent 18FDG PET/CT before treatment were evaluated retrospectively for the detection of nodal and distant metastases. Diagnostic accuracy of 18FDG PET/CT in detecting nodal and distant metastases was compared with that of 18FDG PET and conventional imaging (CI). The images were reviewed and a diagnostic consensus was reached by 3 observers. REFERENCE standard was histologic examination in 15 patients and confirmation of an obvious progression in size of the lesions on follow-up examinations. Nodal metastasis was correctly assessed in 48 patients (96%) with PET/CT, in contrast to 43 patients (86%) with PET, and 46 patients (92%) with CI. Diagnostic accuracies of nodal metastasis in 3 modalities were similar. Using PET/CT, distant metastasis was correctly assigned in 43 patients (86%), whereas interpretation based on PET alone or CI revealed distant metastasis in 33 patients (66%) and 35 patients (70%), respectively. Diagnostic accuracy of distant metastasis with PET/CT was significantly higher than that of PET (P=0.002) or CI (P=0.008). False negative results regarding distant metastasis by PET/CT in 7 patients (14%) were caused by subcentimetric lesions (n=4), bone marrow lesion (n=2), and soft tissue lesions (n=1). PET/CT is more accurate and probably more cost-effective than PET alone or CI regarding distant metastasis in pediatric sarcomas.     

Congenital Nasopharyngeal Teratoma in a Neonate

Background

Congenital germ cell tumors are uncommon. The most common site of teratoma is in the sacrococcygeal region. Teratoma arising from the head and neck comprises less than 10% of reported cases and of these, nasopharyngeal lesions are rare. Teratomas are generally benign, and have a well recognized clinical and histopathological entity. We present a case of nasopharyngeal teratoma (NPT) associated with a wide cleft palate.

Case Presentation

A 20 day old female neonate with a teratoma of the nasopharyngeal area, and wide cleft palate was referred to our center. The protruded mass which measured 6×4×3cm, was of soft consistency, blocked the airway, and prevented oral feeding. Preoperative evaluation and imaging was performed and mass was excised 2 days after admission. Pathology revealed a well-differentiated mature solid teratoma (hairy polyp). The patient had no complication in the post-operative period. Cleft palate was surgically repaired when 2 years old. She is now a six year old girl with normal development.

Conclusion

Congenital nasopharyngeal teratomas are usually benign. Surgery is the treatment of choice, and should be undertaken on an urgent basis, especially in a patient who presents with signs and symptoms of airway obstruction.