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Inflammatory myofibroblastic tumor of parotid in infancy—A new entity
International Journal of Pediatric Otorhinolaryngology 77 (2013) 866–868
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Inflammatory myofibroblastic tumor of parotid in infancy—A new entity Anjan Kumar Dhua a,*, Mohit Garg a, Amita Sen a, Devender S. Chauhan b a b
Department of Pediatric Surgery, PGIMER & Dr RML Hospital, New Delhi, India Department of Pathology, PGIMER & Dr RML Hospital, New Delhi, India
A R T I C L E I N F O
A B S T R A C T
Article history: Received 17 October 2012 Received in revised form 13 February 2013 Accepted 24 February 2013 Available online 3 April 2013
Inflammatory myofibroblastic tumor is commonly found in lungs, mesentery or omentum in the younger population. We hereby report a hitherto unreported case of inflammatory myofibroblastic tumor, arising from the parotid gland in an infant. Diagnosis by histopathology and its treatment by superficial parotidectomy is being presented. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Inflammatory myofibroblastic tumor Plasma cell pseudo-tumor Parotid tumor Parotidectomy
1. Introduction Pediatric parotid masses are rare accounting for only 1% of the pediatric neoplasms [1]. Inflammatory myofibroblastic tumor (IMT) is a neoplasm of unknown etiology arising predominantly in the lungs, mesentery, or omentum in the younger population. Herein we present an infant with parotid mass found to be IMT. 2. Case report A female infant presented with slowly increasing right parotid mass since 3 months of age. There were no other symptoms. The swelling was 3 cm 2 cm and non tender. Skin overlying was non erythematous and was glabrous (Fig. 1). It was smooth, well defined and firm in consistency. Few sub centric ipsilateral cervical lymph nodes were palpable. CECT confirmed a solid well circumscribed lesion arising from the superficial lobe of the parotid (Fig. 2). FNAC was suggestive of a spindle cell mesenchymal lesion. A superficial parotidectomy was performed. The intraoperative findings corroborated with the CT findings and a plane superficial to the facial nerve was confidently developed. The patient made an uneventful postoperative recovery. The HPE report (Fig. 3a) showed tumor tissue composed of spindle cells arranged in sheets, irregular fascicles and present haphazardly in
* Corresponding author at: Room No. 409 PGIMER Building (Adminstration), Baba Kharag Singh Marg, New Delhi 110001, India. Tel.: +91 9871568690. E-mail address: [email protected] (A.K. Dhua). 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.02.020
stroma. The cells had mild pleomorphic oval to spindle shaped nuclei and granular chromatin. The stroma was myxoid and had mixed chronic inflammatory infiltrate comprising of lymphocytes, plasma cells and few eosinophils (Fig. 3b). Stroma also showed rich vascularity (Fig. 3c). Immunohistochemistry revealed focal positivity for CD 68 and for SMA (Fig. 4). Desmin and CK were negative. The described findings were suggestive of IMT. After 10 months of follow up patient is doing well and does not have recurrence. 3. Discussion IMT are regarded as tumors with an indeterminate or low malignant potential. IMT is found throughout the body and affects both sexes and occurs in a wide range of age, however IMT arising from the parotid have been described only in adults [2–4]. In the head and neck it has been described to involve orbits [5], larynx, paranasal sinus [6] and oral cavity. Among the salivary glands parotid [2] is the predominant site for IMT, though one report of submandibular gland being the site of IMT was described by Kojima et al. [7]. The exact etiology of the IMT is not known. Innumerable synonyms (plasma cell granuloma, plasma cell pseudotumor, and inflammatory myofibrohistiocytic proliferation) highlights the fact that this pathology is poorly understood. In the Armed Forces Institute of Pathology (AFIP) Series 4 Atlas of Tumor Pathology, fascicle 9, Tumors of the Salivary Glands published in 2008 [8], the term IMT was used to describe this pathology and was included in the chapter on Non-lymphoid Mesenchymal Neoplasms. Clinical presentation is usually that of a progressively enlarging space occupying lesion with the symptoms
[(Fig._1)TD$IG]
[(Fig._2)TD$IG]
A.K. Dhua et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 866–868
867
Fig. 2. CECT showing a well defined lesion arising from right parotid with preserved normal parotid in deeper aspect (arrow).
Fig. 1. Swelling below right ear and lifting the ear lobe.
depending on the site of origin of the tumor. As far as salivary glands are concerned they manifest as a hard slowly growing mass without involvement of the facial nerve [9]. Diagnosis is generally made by exclusion and biopsy is confirmatory. FNAC has also been described to clinch the diagnosis in one report [3]. Histologically, the lesions contain four essential components: histiocytes, myofibroblasts, plasma cells and lymphocytes. They
[(Fig._3)TD$IG]
are composed of spindle shaped myofibroblasts accompanied by an inflammatory infiltrate. The spindle and stellate-shaped cells are often arranged in a storiform or fascicular pattern in a myxoid to hyaline stroma that typically contains focal to diffuse collections of lymphocytes, plasma cells, histiocytes, and scattered eosinophils. Immunoreactivity to smooth muscle actin (SMA) and muscle specific actin in spindled cells is evident in nearly all tumors; however reactivity can vary from focal to diffuse. They usually are also reactive toward Vimentin as well as KP1 (CD68) [10,3]. Desmin and cytokeratin reactivity are less frequent. Our case also behaved similar to the previously described cases in terms of
Fig. 3. (a) Low power photomicrograph demonstrating spindle cells arranged in sheets, irregular fascicles present haphazardly in stroma. (Hematoxylin & eosin 10). (b) Mixed chronic inflammatory infiltrate interspersed by vessels (arrow). (Hematoxylin & eosin 40). (c) Spindle shaped and rounded cells with abundant cytoplasm and vesicular nuclei. Plasma cells and lymphocytes are scattered throughout the field. (Hematoxylin & eosin 100).
[(Fig._4)TD$IG]
868
A.K. Dhua et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 866–868
Fig. 4. Immunohistochemistry staining showed focal positivity for CD 68 (bold arrow) and for SMA (arrow head) 40.
immunoreactivity therefore based on the histopathological and immunohistochemical findings a diagnosis of IMT was made. Recurrences and metastasis have been described in IMT arising in other parts of body [11] but not in parotid. There is no consensus on the method of management though various modalities have been found to effective. Often, complete surgical excision is both diagnostic and therapeutic. Other therapeutic modalities for IMT are radiotherapy, chemotherapy, azathioprine, indomethacin and steroids. Steroids rapidly reduce the symptoms caused by the edema, but their effect on the primary process has not been proven. Despite this overall ambiguity in management, the literature is uniform with regards to parotid gland where surgical excision has been curative [2,4]. We chose to perform a superficial parotidectomy as the radiological studies clearly depicted that the deep lobe was not involved and this was proved intraoperatively as well. Our case is remarkable because although the histology and immunohistochemistry pointed strongly toward IMT, the age of the patient was highly against this diagnosis. An exhaustive search of the English literature did not reveal any reports of IMT in infancy and all such reported cases were past middle age. Sethi et al. have reported a 12-year-old child with post aural IMT as a complication of trauma and this is the only report having some degree of resemblance to the current case [12]. Thus, it is our belief that this would be the first case of IMT to be reported in an infant.
4. Conclusion The purpose of this case presentation is to highlight that pediatric parotid tumor can have varying pathologies and that IMT must also be included in the differential diagnosis of parotid gland masses in pediatric age group. Preoperative radiological studies can guide whether a superficial or total parotidectomy is to be performed. Adequacy of treatment would however warrant a lifelong follow-up.
Funding None. Conflict of interest None. References [1] C.J. Garcia, P.A. Flores, J.D. Arce, B. Chuaqui, D.S. Schwartz, Ultrasonography in the study of salivary gland lesions in children, Pediatr. Radiol. 28 (1998) 418–425. [2] S.B. Williams, R.D. Foss, G.L. Ellis, Inflammatory pseudotumors of the major salivary glands. Clinicopathologic and immunohistochemical analysis of six cases, Am. J. Surg. Pathol. 16 (1992) 896–902. [3] S. Rahimi, B. Mafera, M.G. Vigili, Inflammatory pseudotumor of the parotid gland: report of a case with fine needle aspiration cytology, Acta Cytol. 48 (2004) 574– 576. [4] S. Van Weert, J.J. Manni, A. Driessen, Inflammatory myofibroblastic tumor of the parotid gland: case report and review of the literature, Acta Otolaryngol. 125 (2005) 433–437. [5] D. Jacobs, S. Galetta, Diagnosis and management of orbital pseudotumor, Curr. Opin. Ophtalmol. 13 (2002) 347–351. [6] P. Escobar Sanz-Dranguet, F.J. Marquez Dorsch, J. Sanabia Brassart, R. Gutierrez Fonseca, J.M. Villacampa Auba, G. Postormelo, et al., Inflammatory pseudotumor of paranasal sinuses, Acta Otorrinolaringol. Esp. 53 (2002) 135–138. [7] M. Kojima, S. Nakamura, H. Itoh, T. Suchi, N. Masawa, Inflammatory pseudotumor of the submandibular gland: report of a case presenting with autoimmune disease-like clinical manifestations, Lab. Med. 125 (2001) 1095–1097. [8] G.L. Ellis, What’s new in the AFIP fascicle on salivary gland tumors: a few highlights from the 4th Series Atlas, Head Neck Pathol. 3 (September (3)) (2009) 225–230. [9] D. Hellı´n Meseguer, E. Merino Ga´lvez, M. Gil Ve´lez, A. Pastor Mas, J. Ruı´z Macı´a, J.E. Herna´ndez, Pseudotumor Inflamatorio de gla´ndula paro´tida, Acta Otorinolaryngol. Iber. Am. 23 (1996) 631–640 (in Spanish). [10] J.K. Brooks, N.G. Nikitakis, B.F. Frankel, J.C. Papadimitriou, J.J. Sauk, Oral inflammatory myofibroblastic tumor demonstrating ALK, p53, MDM2, CDK4, pRb, and Ki-67 immunoreactivity in an elderly patient, Oral. Surg. Oral. Med. Oral. Pathol. Oral. Radiol. Endod. 99 (June (6)) (2005) 716–726. [11] C.M. Coffin, J.L. Hornick, C.D. Fletcher, Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases, Am. J. Surg. Pathol. 31 (2007) 509–520. [12] A. Sethi, V. Malhotra, D. Sethi, S. Nigam, Postaural inflammatory pseudotumor: an extremely unusual complication of trauma in a child, Ear Nose Throat J. 90 (March (3)) (2011) 108–111.
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Inflammatory myofibroblastic tumor of parotid in infancy—A new entity Anjan Kumar Dhua a,*, Mohit Garg a, Amita Sen a, Devender S. Chauhan b a b
Department of Pediatric Surgery, PGIMER & Dr RML Hospital, New Delhi, India Department of Pathology, PGIMER & Dr RML Hospital, New Delhi, India
A R T I C L E I N F O
A B S T R A C T
Article history: Received 17 October 2012 Received in revised form 13 February 2013 Accepted 24 February 2013 Available online 3 April 2013
Inflammatory myofibroblastic tumor is commonly found in lungs, mesentery or omentum in the younger population. We hereby report a hitherto unreported case of inflammatory myofibroblastic tumor, arising from the parotid gland in an infant. Diagnosis by histopathology and its treatment by superficial parotidectomy is being presented. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Inflammatory myofibroblastic tumor Plasma cell pseudo-tumor Parotid tumor Parotidectomy
1. Introduction Pediatric parotid masses are rare accounting for only 1% of the pediatric neoplasms [1]. Inflammatory myofibroblastic tumor (IMT) is a neoplasm of unknown etiology arising predominantly in the lungs, mesentery, or omentum in the younger population. Herein we present an infant with parotid mass found to be IMT. 2. Case report A female infant presented with slowly increasing right parotid mass since 3 months of age. There were no other symptoms. The swelling was 3 cm 2 cm and non tender. Skin overlying was non erythematous and was glabrous (Fig. 1). It was smooth, well defined and firm in consistency. Few sub centric ipsilateral cervical lymph nodes were palpable. CECT confirmed a solid well circumscribed lesion arising from the superficial lobe of the parotid (Fig. 2). FNAC was suggestive of a spindle cell mesenchymal lesion. A superficial parotidectomy was performed. The intraoperative findings corroborated with the CT findings and a plane superficial to the facial nerve was confidently developed. The patient made an uneventful postoperative recovery. The HPE report (Fig. 3a) showed tumor tissue composed of spindle cells arranged in sheets, irregular fascicles and present haphazardly in
* Corresponding author at: Room No. 409 PGIMER Building (Adminstration), Baba Kharag Singh Marg, New Delhi 110001, India. Tel.: +91 9871568690. E-mail address: [email protected] (A.K. Dhua). 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.02.020
stroma. The cells had mild pleomorphic oval to spindle shaped nuclei and granular chromatin. The stroma was myxoid and had mixed chronic inflammatory infiltrate comprising of lymphocytes, plasma cells and few eosinophils (Fig. 3b). Stroma also showed rich vascularity (Fig. 3c). Immunohistochemistry revealed focal positivity for CD 68 and for SMA (Fig. 4). Desmin and CK were negative. The described findings were suggestive of IMT. After 10 months of follow up patient is doing well and does not have recurrence. 3. Discussion IMT are regarded as tumors with an indeterminate or low malignant potential. IMT is found throughout the body and affects both sexes and occurs in a wide range of age, however IMT arising from the parotid have been described only in adults [2–4]. In the head and neck it has been described to involve orbits [5], larynx, paranasal sinus [6] and oral cavity. Among the salivary glands parotid [2] is the predominant site for IMT, though one report of submandibular gland being the site of IMT was described by Kojima et al. [7]. The exact etiology of the IMT is not known. Innumerable synonyms (plasma cell granuloma, plasma cell pseudotumor, and inflammatory myofibrohistiocytic proliferation) highlights the fact that this pathology is poorly understood. In the Armed Forces Institute of Pathology (AFIP) Series 4 Atlas of Tumor Pathology, fascicle 9, Tumors of the Salivary Glands published in 2008 [8], the term IMT was used to describe this pathology and was included in the chapter on Non-lymphoid Mesenchymal Neoplasms. Clinical presentation is usually that of a progressively enlarging space occupying lesion with the symptoms
[(Fig._1)TD$IG]
[(Fig._2)TD$IG]
A.K. Dhua et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 866–868
867
Fig. 2. CECT showing a well defined lesion arising from right parotid with preserved normal parotid in deeper aspect (arrow).
Fig. 1. Swelling below right ear and lifting the ear lobe.
depending on the site of origin of the tumor. As far as salivary glands are concerned they manifest as a hard slowly growing mass without involvement of the facial nerve [9]. Diagnosis is generally made by exclusion and biopsy is confirmatory. FNAC has also been described to clinch the diagnosis in one report [3]. Histologically, the lesions contain four essential components: histiocytes, myofibroblasts, plasma cells and lymphocytes. They
[(Fig._3)TD$IG]
are composed of spindle shaped myofibroblasts accompanied by an inflammatory infiltrate. The spindle and stellate-shaped cells are often arranged in a storiform or fascicular pattern in a myxoid to hyaline stroma that typically contains focal to diffuse collections of lymphocytes, plasma cells, histiocytes, and scattered eosinophils. Immunoreactivity to smooth muscle actin (SMA) and muscle specific actin in spindled cells is evident in nearly all tumors; however reactivity can vary from focal to diffuse. They usually are also reactive toward Vimentin as well as KP1 (CD68) [10,3]. Desmin and cytokeratin reactivity are less frequent. Our case also behaved similar to the previously described cases in terms of
Fig. 3. (a) Low power photomicrograph demonstrating spindle cells arranged in sheets, irregular fascicles present haphazardly in stroma. (Hematoxylin & eosin 10). (b) Mixed chronic inflammatory infiltrate interspersed by vessels (arrow). (Hematoxylin & eosin 40). (c) Spindle shaped and rounded cells with abundant cytoplasm and vesicular nuclei. Plasma cells and lymphocytes are scattered throughout the field. (Hematoxylin & eosin 100).
[(Fig._4)TD$IG]
868
A.K. Dhua et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 866–868
Fig. 4. Immunohistochemistry staining showed focal positivity for CD 68 (bold arrow) and for SMA (arrow head) 40.
immunoreactivity therefore based on the histopathological and immunohistochemical findings a diagnosis of IMT was made. Recurrences and metastasis have been described in IMT arising in other parts of body [11] but not in parotid. There is no consensus on the method of management though various modalities have been found to effective. Often, complete surgical excision is both diagnostic and therapeutic. Other therapeutic modalities for IMT are radiotherapy, chemotherapy, azathioprine, indomethacin and steroids. Steroids rapidly reduce the symptoms caused by the edema, but their effect on the primary process has not been proven. Despite this overall ambiguity in management, the literature is uniform with regards to parotid gland where surgical excision has been curative [2,4]. We chose to perform a superficial parotidectomy as the radiological studies clearly depicted that the deep lobe was not involved and this was proved intraoperatively as well. Our case is remarkable because although the histology and immunohistochemistry pointed strongly toward IMT, the age of the patient was highly against this diagnosis. An exhaustive search of the English literature did not reveal any reports of IMT in infancy and all such reported cases were past middle age. Sethi et al. have reported a 12-year-old child with post aural IMT as a complication of trauma and this is the only report having some degree of resemblance to the current case [12]. Thus, it is our belief that this would be the first case of IMT to be reported in an infant.
4. Conclusion The purpose of this case presentation is to highlight that pediatric parotid tumor can have varying pathologies and that IMT must also be included in the differential diagnosis of parotid gland masses in pediatric age group. Preoperative radiological studies can guide whether a superficial or total parotidectomy is to be performed. Adequacy of treatment would however warrant a lifelong follow-up.
Funding None. Conflict of interest None. References [1] C.J. Garcia, P.A. Flores, J.D. Arce, B. Chuaqui, D.S. Schwartz, Ultrasonography in the study of salivary gland lesions in children, Pediatr. Radiol. 28 (1998) 418–425. [2] S.B. Williams, R.D. Foss, G.L. Ellis, Inflammatory pseudotumors of the major salivary glands. Clinicopathologic and immunohistochemical analysis of six cases, Am. J. Surg. Pathol. 16 (1992) 896–902. [3] S. Rahimi, B. Mafera, M.G. Vigili, Inflammatory pseudotumor of the parotid gland: report of a case with fine needle aspiration cytology, Acta Cytol. 48 (2004) 574– 576. [4] S. Van Weert, J.J. Manni, A. Driessen, Inflammatory myofibroblastic tumor of the parotid gland: case report and review of the literature, Acta Otolaryngol. 125 (2005) 433–437. [5] D. Jacobs, S. Galetta, Diagnosis and management of orbital pseudotumor, Curr. Opin. Ophtalmol. 13 (2002) 347–351. [6] P. Escobar Sanz-Dranguet, F.J. Marquez Dorsch, J. Sanabia Brassart, R. Gutierrez Fonseca, J.M. Villacampa Auba, G. Postormelo, et al., Inflammatory pseudotumor of paranasal sinuses, Acta Otorrinolaringol. Esp. 53 (2002) 135–138. [7] M. Kojima, S. Nakamura, H. Itoh, T. Suchi, N. Masawa, Inflammatory pseudotumor of the submandibular gland: report of a case presenting with autoimmune disease-like clinical manifestations, Lab. Med. 125 (2001) 1095–1097. [8] G.L. Ellis, What’s new in the AFIP fascicle on salivary gland tumors: a few highlights from the 4th Series Atlas, Head Neck Pathol. 3 (September (3)) (2009) 225–230. [9] D. Hellı´n Meseguer, E. Merino Ga´lvez, M. Gil Ve´lez, A. Pastor Mas, J. Ruı´z Macı´a, J.E. Herna´ndez, Pseudotumor Inflamatorio de gla´ndula paro´tida, Acta Otorinolaryngol. Iber. Am. 23 (1996) 631–640 (in Spanish). [10] J.K. Brooks, N.G. Nikitakis, B.F. Frankel, J.C. Papadimitriou, J.J. Sauk, Oral inflammatory myofibroblastic tumor demonstrating ALK, p53, MDM2, CDK4, pRb, and Ki-67 immunoreactivity in an elderly patient, Oral. Surg. Oral. Med. Oral. Pathol. Oral. Radiol. Endod. 99 (June (6)) (2005) 716–726. [11] C.M. Coffin, J.L. Hornick, C.D. Fletcher, Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases, Am. J. Surg. Pathol. 31 (2007) 509–520. [12] A. Sethi, V. Malhotra, D. Sethi, S. Nigam, Postaural inflammatory pseudotumor: an extremely unusual complication of trauma in a child, Ear Nose Throat J. 90 (March (3)) (2011) 108–111.