G Model REMNIM-643; No. of Pages 3
ARTICLE IN PRESS Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
Clinical note
Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature E. Ciftci a,∗ , U. Demirsoy b , Y. Anik c , G. Gorur a , F. Corapcioglu b , H. Demir a a
Department of Nuclear Medicine, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey Department of Pediatric Oncology, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey c Department of Radiology, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey b
a r t i c l e
i n f o
Article history: Received 6 July 2014 Accepted 30 August 2014 Available online xxx Keywords: NUT midline carcinoma 18 F-FDG PET/CT Children Response evaluation
a b s t r a c t NUT midline carcinoma (NMC) is a newly defined and lethal cancer with aggressive course. It mostly affects children and young adults. Diagnosis is confirmed with the evidence of BRD4-NUT mutation on the chromosome 15q14 by fluorescence in situ hybridization. Use of 18 F-FDG PET/CT in NMC patients is very limited in the literature. In this report, we describe a 7-year-old boy with the diagnosis of NMC who was scanned with 18 F-FDG PET/CT for staging and treatment response evaluation after the chemotherapy. It was disseminated and had moderate FDG avidity in the initial scan and showed progression after 4 cycles of chemotherapy. We also reviewed the literature related to 18 F-FDG PET/CT in staging and assessment of chemotherapy response of NMC. © 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
Estadificación y evaluación de la respuesta a quimioterapia neoadyuvante con 18 ˜ presentación F-FDG PET/TAC en carcinoma de línea media – NUT en un nino: de un caso y revisión de la bibliografía r e s u m e n Palabras clave: Carcinoma de línea media – NUT 18 F-FDG PET/TAC ˜ Ninos Evaluación de la respuesta
El Carcinoma de línea media – NUT (NMC), es un cáncer letal recientemente definido con un curso agresivo. ˜ y jóvenes adultos. El diagnóstico se confirma con la evidencia de la mutación de Afecta sobre todo a ninos BRD4-NUT en el cromosoma 15q14 mediante hibridación fluorescente in situ. El uso de 18 F-FDG PET/TAC ˜ de 7 anos ˜ en pacientes con NMC está muy limitado en la bibliografía. En este informe describimos un nino con un diagnóstico de NMC que ha sido explorado con 18 F-FDG PET/TAC para la preparación y evaluación de la respuesta a la quimioterapia. El NMC estaba diseminado y mostraba una moderada captación de FDG en una exploración inicial y presentando una progresión después de 4 ciclos de quimioterapia. También analizamos la bibliografía relacionada con 18 F-FDG PET/TAC en la estadificación y evaluación de la repuesta a la quimioterapia del NMC. © 2014 Elsevier España, S.L.U. y SEMNIM. Todos los derechos reservados.
Introduction NUT midline carcinoma (NMC) is a rare, poorly differentiated tumor that is mainly originated from midline of the body, mostly in children and young adults. This tumor has an aggressive clinical course and is usually presented as local advanced and disseminated disease at the time of diagnosis.1,2 NMC genetically results from chromosomal rearrangements of the NUT (nuclear protein in testis) gene on chromosome 15q14 producing aBRD4-NUT fusion
∗ Corresponding author. E-mail addresses: [email protected], [email protected] (E. Ciftci), [email protected] (U. Demirsoy), [email protected] (Y. Anik), [email protected] (G. Gorur), [email protected] (F. Corapcioglu), [email protected] (H. Demir).
oncogene. This oncogene causes uncontrolled cell growth by blocking epithelial differentiation. There are limited case reports showing the benefit of using FDG PET/CT for staging and evaluation of treatment response in NMC.1–4 Herein, we report a case of a 7-year-old boy with disseminated metastatic disease of NMC evaluated by FDG PET/CT at the time of diagnosis and after therapy. Case report A 7-year-old boy initially presented to his primary care physician with a 2-month history of coughing, sweating and anorexia. He was treated with oral antibiotics without resolution of his symptoms and referred to our hospital’s pediatric oncology clinic. On physical examination, he was pale and had left-side micro cervical lymph nodes as well as decreased breath sounds in the right chest. The remainder of the physical examination yielded unremarkable
http://dx.doi.org/10.1016/j.remn.2014.08.007 2253-654X/© 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
G Model REMNIM-643; No. of Pages 3 2
ARTICLE IN PRESS E. Ciftci et al. / Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
Fig. 1. PET/CT images at initial staging: MIP (maximum intensity projection) image of PET (a), coronal images of PET (b) and fused PET/CT (c) revealed moderated increased uptake on the left cervical, mediastinal, abdominopelvic adenopathies, central mass in the right hemithorax, with widespread liver and bone metastases in the right humerus, right scapula, left clavicula, pelvis and bilateral lower extremities.
findings. There was no family history of malignancy and no previous medical history. Bone marrow aspiration and biopsy were normal. Chest X-ray demonstrated complete opacification of the lower region of right hemithorax with right hilar attenuation and mediastinal fullness. Thorax CT revealed multiple lymphadenopathies located at right paratracheal, subcarinal, precarinal, right hilar, paracardiac inseparable from the right atrium, with occlusion of the right middle lobe bronchus that lead to middle lobe atelectasis and infiltration at right lower lobe. PET/CT (Discovery 690, GE Healthcare, WI, USA) was performed subsequently at 1 h after the injection of 14.5 MBq 18 F FDG in fasting state. It demonstrated FDGavid left cervical, mediastinal, abdominopelvic adenopathies with maximum standardized uptake values (SUVmax) ranging from 2.7 to 5.4, central mass in the right hemithorax (SUVmax: 5.8) with widespread liver (SUVmax: 3.7) and lytic/sclerotic bone metastases in the right humerus, right scapula, left clavicula, pelvis and bilateral lower extremities (SUVmax: 6.6) (Fig. 1). Since the patient represented with bulky disease in abdominal region, a biopsy of a mesenteric lymph node was performed which revealed an undifferentiated carcinoma negative for lymphoma and sarcoma markers with immunohistochemistry suggesting solid component of blastomatous tumor. Emergent chemotherapy was initiated that is effective against both NUT midline carcinoma and germ cell tumor, while sections were sent for fluorescent in situ hybridization (FISH) analysis at Brigham and Women’s Hospital (Boston, MA, USA). The results of which were BRD4 rearrangement, consisted with a BRD4-NUT fusion and it was consistent with NUT midline carcinoma.
Fig. 2. FDG PET/CT after 4 cycles of chemotherapy: MIP (maximum intensity projection) image of PET (a), coronal images of PET (b) and fused PET/CT (c) showed increasing in metabolic activity of initially described cervical, mediastinal, abdominopelvic adenopathies, significant metabolic progression of central mass in the right hemithorax. Besides liver lesions disappeared and multiple osseous metastatic lesions showed mixed response, new FDG-avid lesions were observed at left humerus, aortopulmonary window of mediastinum and paracolic region at the midline of the abdomen.
FDG PET/CT following 4 cycles chemotherapy was performed with the same protocol of the initial scan, showed increasing in metabolic activity of initially described cervical, mediastinal, abdominopelvic adenopathies, significant metabolic progression of central mass in the right hemithorax. Besides liver lesions disappeared and multiple osseous metastatic lesions showed mixed response, new FDG-avid lesions were observed at left humerus, aortopulmonary window of mediastinum and paracolic region at the midline of the abdomen (Fig. 2). MRI findings confirmed FDG PET/CT findings regarding progressive abdominal disease. Chemotherapy regimen was changed and concomitant palliative radiotherapy was initiated. Discussion NMC is a rare and newly defined condition that has a lethal clinical course. Because of its rarity, lack of characteristic clinical and pathological features, many cases of NMC may be undefined and may be overlapped with other high-grade carcinomas by means of morphologic/histologic appearance.5 And also additional differential considerations included germ cell tumor, stage IV neuroblastoma, hepatoblastoma, sarcomatous tumors like rhabdomyosarcoma and lymphoma.1,2,6 Karyotyping or fluorescent in situ hybridization should be performed for definite diagnosis. There is very limited literature about imaging appearance of NMC. We are aware of only 4 publications regarding the appearance
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
G Model REMNIM-643; No. of Pages 3
ARTICLE IN PRESS E. Ciftci et al. / Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
on FDG PET/CT of NMC. Niederkohr et al.1 reported a 13-year-old girl presented with lung lesion with the presence of a BRD4NUT mutation. They used FDG PET/CT in assessing response to chemotherapy. After the initial favorable response of chemotherapy showed with repeated FDG PET/CT scans, they demonstrated the progression in hypermetabolic metastatic osseous lesions all of which were highly FDG-avid. The authors concluded that, the use of FDG PET/CT in the detection, staging and restaging of this disease is favorable. High FDG-avidity of NMC was seen in that case because of hypermetabolic character of this aggressive tumor. But in our case, moderate FDG avidity was observed at diagnosis and higher FDG avidity of tumor lesions occurred during progression despite chemotherapy. Rutt et al.3 reported a case report of NMC in an 8-year-old girl that was misdiagnosed as tonsillar abscess. After the treatment with oral antibiotics without resolution of her symptoms, malignancy was suspected. The diagnosis of NMC was confirmed by FISH after cervical lymph node excisional biopsy. While the initial FDG PET scan demonstrated only multiple FDG-avid lymph nodes in bilateral cervical chains with (SUVmax) ranging from 6.8 to 13.6, follow-up FDG PET scan showed regression of local cervical disease but progression of the tumor with multiple hypermetabolic metastatic osseous lesions despite the chemoradiotherapy. Although our patient also presented with nonspecific signs and symptoms such as pallor, coughing, sweating and anorexia those are commonly seen in benign illnesses during childhood, malignancy was clinically suspected and disseminated disease was observed by FDG PET/CT at initial presentation. FDG PET/CT helped us not only showing disseminated disease but also guiding for biopsy localization from abdominal region presented as bulky disease with highest FDG uptake. As in this case, our patient also demonstrated mixed response to the chemotherapy. Rosenbaum et al.4 reported a 17 year-old boy with the diagnosis of NMC at soft tissue mass in the posterior mediastinum with bulky thoracic and cervical adenopathy. After CT examination of the chest, abdomen and pelvis at initial staging, they performed FDG PET/CT for assessment of response to three cycles of chemotherapy. While the primary tumor responded to the chemotherapy; FDG PET/CT showed accompanying widespread lytic bone metastases as in previous case. Polsani et al.2 retrospectively reviewed 3 cases of NMC, only one of which had FDG PET/CT imaging. The authors reported a 2 yearold boy with NMC demonstrated as poorly defined mass invading the left liver lobe, pancreatic head and vessels with bilateral lower
3
lobe lung nodules observed at initial abdominopelvic CT. They used FDG PET/CT in restaging of the disease after the chemotherapy and showed progression of primary tumor and newly defined metastases in the lung, mediastinum and omentum with high FDGavidity. Among these 4 cases, 3 were children and one was young adult. Three cases’ primary lesions responded the chemotherapy at interim FDG PET/CT with rapid progression of cancer thereafter. The first two cases mentioned above1,3 which is initially staged with PET/CT, tumors showed high FDG-avidity at the time of diagnosis. But our case has moderate FDG avidity at initial stage. All cases1–4 reported increased hypermetabolism despite the chemotherapy similar to our case. With this case report, we believe that our contribution to the literature is showing moderate FDG uptake of NMC at initial stage and taking advantage of the PET/CT to guide for biopsy localization. To the best of our knowledge, this is the 5th case of NMC in which FDG PET/CT was used in the staging and assessing the response of chemotherapy. In conclusion, NMC is a highly aggressive cancer. There is a very limited data regarding the imaging findings of NMC. With this case review, we have demonstrated that moderate to high FDG avidity may be presented at NMC and also benefit of FDG PET/CT usage in staging, biopsy localization and restaging of NMC is noteworthy. Acknowledgement We thank Dr Christopher A. French for reviewing the pathology specimen and confirming the diagnosis of NUT midline carcinoma. References 1. Niederkohr RD, Cameron MJ, French CA. FDG PET/CT imaging of NUT midline carcinoma. Clin Nucl Med. 2011;36:124–6. 2. Polsani A, Braithwaite KA, Alazraki AL, Abramowsky C, Shehata BM. NUT midline carcinoma: an imaging case series and review of literature. Pediatr Radiol. 2012;42:205–10. 3. Rutt AL, Poulik J, Siddiqui AH, Konski A, Kalaf M, Madgy DN, et al. NUT midline carcinoma mimicking tonsillitis in an eight-year-old girl. Ann Otol Rhinol Laryngol. 2011;120:546–9. 4. Rosenbaum DG, Teruya-Feldstein J, Price AP, Meyers P, Abramson S. Radiologic features of NUT midline carcinoma in an adolescent. Pediatr Radiol. 2012;42:249–52. 5. Bellizzi AM, Bruzzi C, French CA, Stelow EB. The cytologic features of NUT midline carcinoma. Cancer. 2009;117:508–15. 6. Shehata BM, Steelman CK, Abramowsky CR, Olson TA, French CA, Saxe DF, et al. NUT midline carcinoma in a newborn with multiorgan disseminated tumor and a 2-year-old with a pancreatic/hepatic primary. Pediatr Dev Pathol. 2010;13:481–5.
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
ARTICLE IN PRESS Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
Clinical note
Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature E. Ciftci a,∗ , U. Demirsoy b , Y. Anik c , G. Gorur a , F. Corapcioglu b , H. Demir a a
Department of Nuclear Medicine, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey Department of Pediatric Oncology, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey c Department of Radiology, Faculty of Medicine, University of Kocaeli, Kocaeli, Turkey b
a r t i c l e
i n f o
Article history: Received 6 July 2014 Accepted 30 August 2014 Available online xxx Keywords: NUT midline carcinoma 18 F-FDG PET/CT Children Response evaluation
a b s t r a c t NUT midline carcinoma (NMC) is a newly defined and lethal cancer with aggressive course. It mostly affects children and young adults. Diagnosis is confirmed with the evidence of BRD4-NUT mutation on the chromosome 15q14 by fluorescence in situ hybridization. Use of 18 F-FDG PET/CT in NMC patients is very limited in the literature. In this report, we describe a 7-year-old boy with the diagnosis of NMC who was scanned with 18 F-FDG PET/CT for staging and treatment response evaluation after the chemotherapy. It was disseminated and had moderate FDG avidity in the initial scan and showed progression after 4 cycles of chemotherapy. We also reviewed the literature related to 18 F-FDG PET/CT in staging and assessment of chemotherapy response of NMC. © 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
Estadificación y evaluación de la respuesta a quimioterapia neoadyuvante con 18 ˜ presentación F-FDG PET/TAC en carcinoma de línea media – NUT en un nino: de un caso y revisión de la bibliografía r e s u m e n Palabras clave: Carcinoma de línea media – NUT 18 F-FDG PET/TAC ˜ Ninos Evaluación de la respuesta
El Carcinoma de línea media – NUT (NMC), es un cáncer letal recientemente definido con un curso agresivo. ˜ y jóvenes adultos. El diagnóstico se confirma con la evidencia de la mutación de Afecta sobre todo a ninos BRD4-NUT en el cromosoma 15q14 mediante hibridación fluorescente in situ. El uso de 18 F-FDG PET/TAC ˜ de 7 anos ˜ en pacientes con NMC está muy limitado en la bibliografía. En este informe describimos un nino con un diagnóstico de NMC que ha sido explorado con 18 F-FDG PET/TAC para la preparación y evaluación de la respuesta a la quimioterapia. El NMC estaba diseminado y mostraba una moderada captación de FDG en una exploración inicial y presentando una progresión después de 4 ciclos de quimioterapia. También analizamos la bibliografía relacionada con 18 F-FDG PET/TAC en la estadificación y evaluación de la repuesta a la quimioterapia del NMC. © 2014 Elsevier España, S.L.U. y SEMNIM. Todos los derechos reservados.
Introduction NUT midline carcinoma (NMC) is a rare, poorly differentiated tumor that is mainly originated from midline of the body, mostly in children and young adults. This tumor has an aggressive clinical course and is usually presented as local advanced and disseminated disease at the time of diagnosis.1,2 NMC genetically results from chromosomal rearrangements of the NUT (nuclear protein in testis) gene on chromosome 15q14 producing aBRD4-NUT fusion
∗ Corresponding author. E-mail addresses: [email protected], [email protected] (E. Ciftci), [email protected] (U. Demirsoy), [email protected] (Y. Anik), [email protected] (G. Gorur), [email protected] (F. Corapcioglu), [email protected] (H. Demir).
oncogene. This oncogene causes uncontrolled cell growth by blocking epithelial differentiation. There are limited case reports showing the benefit of using FDG PET/CT for staging and evaluation of treatment response in NMC.1–4 Herein, we report a case of a 7-year-old boy with disseminated metastatic disease of NMC evaluated by FDG PET/CT at the time of diagnosis and after therapy. Case report A 7-year-old boy initially presented to his primary care physician with a 2-month history of coughing, sweating and anorexia. He was treated with oral antibiotics without resolution of his symptoms and referred to our hospital’s pediatric oncology clinic. On physical examination, he was pale and had left-side micro cervical lymph nodes as well as decreased breath sounds in the right chest. The remainder of the physical examination yielded unremarkable
http://dx.doi.org/10.1016/j.remn.2014.08.007 2253-654X/© 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
G Model REMNIM-643; No. of Pages 3 2
ARTICLE IN PRESS E. Ciftci et al. / Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
Fig. 1. PET/CT images at initial staging: MIP (maximum intensity projection) image of PET (a), coronal images of PET (b) and fused PET/CT (c) revealed moderated increased uptake on the left cervical, mediastinal, abdominopelvic adenopathies, central mass in the right hemithorax, with widespread liver and bone metastases in the right humerus, right scapula, left clavicula, pelvis and bilateral lower extremities.
findings. There was no family history of malignancy and no previous medical history. Bone marrow aspiration and biopsy were normal. Chest X-ray demonstrated complete opacification of the lower region of right hemithorax with right hilar attenuation and mediastinal fullness. Thorax CT revealed multiple lymphadenopathies located at right paratracheal, subcarinal, precarinal, right hilar, paracardiac inseparable from the right atrium, with occlusion of the right middle lobe bronchus that lead to middle lobe atelectasis and infiltration at right lower lobe. PET/CT (Discovery 690, GE Healthcare, WI, USA) was performed subsequently at 1 h after the injection of 14.5 MBq 18 F FDG in fasting state. It demonstrated FDGavid left cervical, mediastinal, abdominopelvic adenopathies with maximum standardized uptake values (SUVmax) ranging from 2.7 to 5.4, central mass in the right hemithorax (SUVmax: 5.8) with widespread liver (SUVmax: 3.7) and lytic/sclerotic bone metastases in the right humerus, right scapula, left clavicula, pelvis and bilateral lower extremities (SUVmax: 6.6) (Fig. 1). Since the patient represented with bulky disease in abdominal region, a biopsy of a mesenteric lymph node was performed which revealed an undifferentiated carcinoma negative for lymphoma and sarcoma markers with immunohistochemistry suggesting solid component of blastomatous tumor. Emergent chemotherapy was initiated that is effective against both NUT midline carcinoma and germ cell tumor, while sections were sent for fluorescent in situ hybridization (FISH) analysis at Brigham and Women’s Hospital (Boston, MA, USA). The results of which were BRD4 rearrangement, consisted with a BRD4-NUT fusion and it was consistent with NUT midline carcinoma.
Fig. 2. FDG PET/CT after 4 cycles of chemotherapy: MIP (maximum intensity projection) image of PET (a), coronal images of PET (b) and fused PET/CT (c) showed increasing in metabolic activity of initially described cervical, mediastinal, abdominopelvic adenopathies, significant metabolic progression of central mass in the right hemithorax. Besides liver lesions disappeared and multiple osseous metastatic lesions showed mixed response, new FDG-avid lesions were observed at left humerus, aortopulmonary window of mediastinum and paracolic region at the midline of the abdomen.
FDG PET/CT following 4 cycles chemotherapy was performed with the same protocol of the initial scan, showed increasing in metabolic activity of initially described cervical, mediastinal, abdominopelvic adenopathies, significant metabolic progression of central mass in the right hemithorax. Besides liver lesions disappeared and multiple osseous metastatic lesions showed mixed response, new FDG-avid lesions were observed at left humerus, aortopulmonary window of mediastinum and paracolic region at the midline of the abdomen (Fig. 2). MRI findings confirmed FDG PET/CT findings regarding progressive abdominal disease. Chemotherapy regimen was changed and concomitant palliative radiotherapy was initiated. Discussion NMC is a rare and newly defined condition that has a lethal clinical course. Because of its rarity, lack of characteristic clinical and pathological features, many cases of NMC may be undefined and may be overlapped with other high-grade carcinomas by means of morphologic/histologic appearance.5 And also additional differential considerations included germ cell tumor, stage IV neuroblastoma, hepatoblastoma, sarcomatous tumors like rhabdomyosarcoma and lymphoma.1,2,6 Karyotyping or fluorescent in situ hybridization should be performed for definite diagnosis. There is very limited literature about imaging appearance of NMC. We are aware of only 4 publications regarding the appearance
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
G Model REMNIM-643; No. of Pages 3
ARTICLE IN PRESS E. Ciftci et al. / Rev Esp Med Nucl Imagen Mol. 2014;xxx(xx):xxx–xxx
on FDG PET/CT of NMC. Niederkohr et al.1 reported a 13-year-old girl presented with lung lesion with the presence of a BRD4NUT mutation. They used FDG PET/CT in assessing response to chemotherapy. After the initial favorable response of chemotherapy showed with repeated FDG PET/CT scans, they demonstrated the progression in hypermetabolic metastatic osseous lesions all of which were highly FDG-avid. The authors concluded that, the use of FDG PET/CT in the detection, staging and restaging of this disease is favorable. High FDG-avidity of NMC was seen in that case because of hypermetabolic character of this aggressive tumor. But in our case, moderate FDG avidity was observed at diagnosis and higher FDG avidity of tumor lesions occurred during progression despite chemotherapy. Rutt et al.3 reported a case report of NMC in an 8-year-old girl that was misdiagnosed as tonsillar abscess. After the treatment with oral antibiotics without resolution of her symptoms, malignancy was suspected. The diagnosis of NMC was confirmed by FISH after cervical lymph node excisional biopsy. While the initial FDG PET scan demonstrated only multiple FDG-avid lymph nodes in bilateral cervical chains with (SUVmax) ranging from 6.8 to 13.6, follow-up FDG PET scan showed regression of local cervical disease but progression of the tumor with multiple hypermetabolic metastatic osseous lesions despite the chemoradiotherapy. Although our patient also presented with nonspecific signs and symptoms such as pallor, coughing, sweating and anorexia those are commonly seen in benign illnesses during childhood, malignancy was clinically suspected and disseminated disease was observed by FDG PET/CT at initial presentation. FDG PET/CT helped us not only showing disseminated disease but also guiding for biopsy localization from abdominal region presented as bulky disease with highest FDG uptake. As in this case, our patient also demonstrated mixed response to the chemotherapy. Rosenbaum et al.4 reported a 17 year-old boy with the diagnosis of NMC at soft tissue mass in the posterior mediastinum with bulky thoracic and cervical adenopathy. After CT examination of the chest, abdomen and pelvis at initial staging, they performed FDG PET/CT for assessment of response to three cycles of chemotherapy. While the primary tumor responded to the chemotherapy; FDG PET/CT showed accompanying widespread lytic bone metastases as in previous case. Polsani et al.2 retrospectively reviewed 3 cases of NMC, only one of which had FDG PET/CT imaging. The authors reported a 2 yearold boy with NMC demonstrated as poorly defined mass invading the left liver lobe, pancreatic head and vessels with bilateral lower
3
lobe lung nodules observed at initial abdominopelvic CT. They used FDG PET/CT in restaging of the disease after the chemotherapy and showed progression of primary tumor and newly defined metastases in the lung, mediastinum and omentum with high FDGavidity. Among these 4 cases, 3 were children and one was young adult. Three cases’ primary lesions responded the chemotherapy at interim FDG PET/CT with rapid progression of cancer thereafter. The first two cases mentioned above1,3 which is initially staged with PET/CT, tumors showed high FDG-avidity at the time of diagnosis. But our case has moderate FDG avidity at initial stage. All cases1–4 reported increased hypermetabolism despite the chemotherapy similar to our case. With this case report, we believe that our contribution to the literature is showing moderate FDG uptake of NMC at initial stage and taking advantage of the PET/CT to guide for biopsy localization. To the best of our knowledge, this is the 5th case of NMC in which FDG PET/CT was used in the staging and assessing the response of chemotherapy. In conclusion, NMC is a highly aggressive cancer. There is a very limited data regarding the imaging findings of NMC. With this case review, we have demonstrated that moderate to high FDG avidity may be presented at NMC and also benefit of FDG PET/CT usage in staging, biopsy localization and restaging of NMC is noteworthy. Acknowledgement We thank Dr Christopher A. French for reviewing the pathology specimen and confirming the diagnosis of NUT midline carcinoma. References 1. Niederkohr RD, Cameron MJ, French CA. FDG PET/CT imaging of NUT midline carcinoma. Clin Nucl Med. 2011;36:124–6. 2. Polsani A, Braithwaite KA, Alazraki AL, Abramowsky C, Shehata BM. NUT midline carcinoma: an imaging case series and review of literature. Pediatr Radiol. 2012;42:205–10. 3. Rutt AL, Poulik J, Siddiqui AH, Konski A, Kalaf M, Madgy DN, et al. NUT midline carcinoma mimicking tonsillitis in an eight-year-old girl. Ann Otol Rhinol Laryngol. 2011;120:546–9. 4. Rosenbaum DG, Teruya-Feldstein J, Price AP, Meyers P, Abramson S. Radiologic features of NUT midline carcinoma in an adolescent. Pediatr Radiol. 2012;42:249–52. 5. Bellizzi AM, Bruzzi C, French CA, Stelow EB. The cytologic features of NUT midline carcinoma. Cancer. 2009;117:508–15. 6. Shehata BM, Steelman CK, Abramowsky CR, Olson TA, French CA, Saxe DF, et al. NUT midline carcinoma in a newborn with multiorgan disseminated tumor and a 2-year-old with a pancreatic/hepatic primary. Pediatr Dev Pathol. 2010;13:481–5.
Please cite this article in press as: Ciftci E, et al. Staging and evaluation of neoadjuvant chemotherapy response with 18 F-FDG PET/CT in NUT-midline carcinoma in a child: A case report and review of the literature. Rev Esp Med Nucl Imagen Mol. 2014. http://dx.doi.org/10.1016/j.remn.2014.08.007
