Clinical Review & Education
Clinical Challenge | PATHOLOGY
Worsening Headache and Nasal Congestion Ashley E. Kita, BS; Sunita M. Bhuta, MD; Marilene B. Wang, MD
A
B
C
Figure. A, Contrast-enhanced T1-weighted magnetic resonance imaging of a soft-tissue mass within the left ethmoid cavity. B, Central vessel surrounded by lobules of endothelial-lined capillaries (hematoxylin-eosin, original magnification ×20). C, Endothelial cells of the numerous capillaries and the central vessel on CD34 staining (original magnification ×20).
A woman in her 50s presented with an 8-week history of increasing headache, hyposmia, hypogeusia, and nasal congestion. Endoscopic nasopharyngoscopy revealed a large polypoid nasal mass occupying the left nasal roof and completely obstructing the nasal passage. Contrast-enhanced T1-weighted magnetic resonance imaging (MRI) revealed a 1.7 × 3.5 × 2.6-cm Quiz at soft-tissue mass within the left ethmoid cavity, eroding jamaotolaryngology.com through the cribriform plate with adjacent dural enhancement (Figure, A). Intraoperative pathologic examination showed polypoid spindle cell proliferation. Histopathologic findings showed respiratory epithelium with submucosal vascular proliferation with no atypia among the lining endothelial cells. The Figure, B, shows a central vessel surrounded by lobules of endothelial lined capillaries. The Figure, C, highlights the endothelial cells of the numerous capillaries and the central vessel.
jamaotolaryngology.com
WHAT IS YOUR DIAGNOSIS?
A. Juvenile nasopharyngeal angiofibroma B. Lobular capillary hemangioma C. Inverting papilloma D. Respiratory epithelial adenomatoid hamartoma
(Reprinted) JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Purdue University User on 05/21/2015
387
Clinical Review & Education Clinical Challenge
Diagnosis B. Lobular capillary hemangioma
Discussion Nasal lobular capillary hemangiomas, also known as pyogenic granulomas, are benign skin and mucosal lesions of unclear etiology, associated with trauma, pregnancy, and oral contraceptive use. Because they are neither infectious nor granulomatous, the later term has fallen out of favor. Most lobular capillary hemangiomas are found in the oral cavity, with rare occurrence in the nasal cavity. While the exact cause in unknown, pregnant women and individuals with a history of trauma comprise 15% and 12% of reported cases, respectively, suggesting a relationship between these lesions and elevated hormone states and inflammatory changes.1 One study2 suggests that up to 5% of pregnant women may be found to have these lesions. Furthermore, cytogenetic analysis of one individual identified a deletion on the long arm of chromosome 21 as the sole clonal cytogenic abnormality among cells of the lesion, suggesting a neoplastic basis.3 On histologic examination, lesions typically appear grossly as 0.2to 2.5-cm polypoid masses that may have areas of ulceration, although lesions as large as 5 cm have been described.2 Superficial stratified squamous epithelium may contain sites of atrophy of ulceration.4 The lamina propria is filled with lobular proliferations of capillaries lined by thickened endothelial cells and filled with blood.5 Lobules are separated by fibrous septa containing scattered inflammatory cells and thin vessels feeding into larger “feeder vessels” that surround the lobules circumferentially.6 While a surrounding infiltrate of inflammatory cells may be observed, lobules are usually present to make the diagnosis. Endothelial cells stain positive for typical endothelial markers, including CD31, CD34, and factor VIII.4 Other differential diagnoses include angiofibromas, angiomatous polyp, and malignant neoplasms, such as nasopharyngeal carcinoma or nasopharyngeal teratoma.6 Individuals typically present with some combination of epistaxis, congestion, and pain. Visualization reveals a unilateral ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Head and Neck Surgery, University of California, Los Angeles, Los Angeles (Kita, Wang); Department of Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles (Bhuta).
1. Smith SC, Patel RM, Lucas DR, McHugh JB. Sinonasal lobular capillary hemangioma: a clinicopathologic study of 34 cases characterizing potential for local recurrence. Head Neck Pathol. 2013;7(2):129-134.
Corresponding Author: Marilene B. Wang, MD, Department of Head and Neck Surgery, University of California, Los Angeles, 200 Medical Plaza, Ste 550, Los Angeles, CA 90095-6959 ([email protected]).
2. Choudhary S, MacKinnon CA, Morrissey GP, Tan ST. A case of giant nasal pyogenic granuloma gravidarum. J Craniofac Surg. 2005;16(2):319-321.
Section Editor: Edward B. Stelow, MD. Published Online: January 22, 2015. doi:10.1001/jamaoto.2014.3539. Conflict of Interest Disclosures: None reported.
388
mobile, nonpulsatile polypoid mass. Lesions affect individuals of all ages and have no preference with regard sex.6 Sixty-seven percent of lesions involve the septum; 18%, the vestibule; 12%, the turbinate; and 3%, the ethmoid sinus.1 Imaging shows a soft-tissue enhancing mass. Bony destruction is rare.7 Contrast-enhanced computed tomography shows an enhancing mass with a surrounding border of isoattenuation or hypoattenuation. Magnetic resonance imaging shows hypointensity on T1-weighted imaging, hyperintensity on T2-weighted imaging, and enhancement with gadolinium. Imaging has been found to be useful in assessing the vascular nature of these lesions and in assessing the extent of involvement, although often unnecessary for welllocalized lesions of small size.8 Treatment typically involves embolization and excision. Paranasal endoscopic techniques are most common and are often accompanied by further treatment, such as electrocautery, silver nitrate, or absorbable hemostatic agents.1 While small lesions are amenable to endoscopic removal, larger lesions have typically necessitated open craniofacial techniques, with bleeding being the main deterrent. 7 This case demonstrates that even large lesions with intradural extension can be completely resected via endoscopic techniques. While a review 4 performed in 2006 found no recurrence among the 40 cases included, a 2013 review1 of 34 cases found recurrence in up to 42% of individuals with nasal lobular capillary hemangioma. This review found that lesions recur an average of 5.7 months later. Whether this represents a failure to excise the lesion completely or an increase in spontaneous cellular changes among susceptible hosts is unclear. In addition, within adolescents and gravid women, lesions may be observed to gradually regress over a period of weeks without intervention.5 Lobular capillary hemangiomas of the nasal cavity are rare and may be detected later than those of the oral cavity. They should be considered in the presence of any enlarging vascular lesion within the nasal cavities.
3. Truss L, Dobin SM, Donner LR. Deletion (21)(q21.2q22.12) as a sole clonal cytogenetic abnormality in a lobular capillary hemangioma of the nasal cavity. Cancer Genet Cytogenet. 2006;170 (1):69-70.
5. Zarrinneshan AA, Zapanta PE, Wall SJ. Nasal pyogenic granuloma. Otolaryngol Head Neck Surg. 2007;136(1):130-131. 6. Patil P, Singla S, Mane R, Jagdeesh KS. Nasal lobular capillary hemangioma. J Clin Imaging Sci. 2013;3:40. 7. Lombardi D, Galtelli C, Khrais T, Morassi ML, Nicolai P. Giant hypervascular lesion of the sinonasal tract invading the anterior skull base and orbit: a puzzling case. Ann Otol Rhinol Laryngol. 2008;117(9):653-658. 8. Lee DG, Lee SK, Chang HW, et al. CT features of lobular capillary hemangioma of the nasal cavity. AJNR Am J Neuroradiol. 2010;31(4):749-754.
4. Puxeddu R, Berlucchi M, Ledda GP, Parodo G, Farina D, Nicolai P. Lobular capillary hemangioma of the nasal cavity: a retrospective study on 40 patients. Am J Rhinol. 2006;20(4):480-484.
JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Purdue University User on 05/21/2015
jamaotolaryngology.com
Clinical Challenge | PATHOLOGY
Worsening Headache and Nasal Congestion Ashley E. Kita, BS; Sunita M. Bhuta, MD; Marilene B. Wang, MD
A
B
C
Figure. A, Contrast-enhanced T1-weighted magnetic resonance imaging of a soft-tissue mass within the left ethmoid cavity. B, Central vessel surrounded by lobules of endothelial-lined capillaries (hematoxylin-eosin, original magnification ×20). C, Endothelial cells of the numerous capillaries and the central vessel on CD34 staining (original magnification ×20).
A woman in her 50s presented with an 8-week history of increasing headache, hyposmia, hypogeusia, and nasal congestion. Endoscopic nasopharyngoscopy revealed a large polypoid nasal mass occupying the left nasal roof and completely obstructing the nasal passage. Contrast-enhanced T1-weighted magnetic resonance imaging (MRI) revealed a 1.7 × 3.5 × 2.6-cm Quiz at soft-tissue mass within the left ethmoid cavity, eroding jamaotolaryngology.com through the cribriform plate with adjacent dural enhancement (Figure, A). Intraoperative pathologic examination showed polypoid spindle cell proliferation. Histopathologic findings showed respiratory epithelium with submucosal vascular proliferation with no atypia among the lining endothelial cells. The Figure, B, shows a central vessel surrounded by lobules of endothelial lined capillaries. The Figure, C, highlights the endothelial cells of the numerous capillaries and the central vessel.
jamaotolaryngology.com
WHAT IS YOUR DIAGNOSIS?
A. Juvenile nasopharyngeal angiofibroma B. Lobular capillary hemangioma C. Inverting papilloma D. Respiratory epithelial adenomatoid hamartoma
(Reprinted) JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Purdue University User on 05/21/2015
387
Clinical Review & Education Clinical Challenge
Diagnosis B. Lobular capillary hemangioma
Discussion Nasal lobular capillary hemangiomas, also known as pyogenic granulomas, are benign skin and mucosal lesions of unclear etiology, associated with trauma, pregnancy, and oral contraceptive use. Because they are neither infectious nor granulomatous, the later term has fallen out of favor. Most lobular capillary hemangiomas are found in the oral cavity, with rare occurrence in the nasal cavity. While the exact cause in unknown, pregnant women and individuals with a history of trauma comprise 15% and 12% of reported cases, respectively, suggesting a relationship between these lesions and elevated hormone states and inflammatory changes.1 One study2 suggests that up to 5% of pregnant women may be found to have these lesions. Furthermore, cytogenetic analysis of one individual identified a deletion on the long arm of chromosome 21 as the sole clonal cytogenic abnormality among cells of the lesion, suggesting a neoplastic basis.3 On histologic examination, lesions typically appear grossly as 0.2to 2.5-cm polypoid masses that may have areas of ulceration, although lesions as large as 5 cm have been described.2 Superficial stratified squamous epithelium may contain sites of atrophy of ulceration.4 The lamina propria is filled with lobular proliferations of capillaries lined by thickened endothelial cells and filled with blood.5 Lobules are separated by fibrous septa containing scattered inflammatory cells and thin vessels feeding into larger “feeder vessels” that surround the lobules circumferentially.6 While a surrounding infiltrate of inflammatory cells may be observed, lobules are usually present to make the diagnosis. Endothelial cells stain positive for typical endothelial markers, including CD31, CD34, and factor VIII.4 Other differential diagnoses include angiofibromas, angiomatous polyp, and malignant neoplasms, such as nasopharyngeal carcinoma or nasopharyngeal teratoma.6 Individuals typically present with some combination of epistaxis, congestion, and pain. Visualization reveals a unilateral ARTICLE INFORMATION
REFERENCES
Author Affiliations: Department of Head and Neck Surgery, University of California, Los Angeles, Los Angeles (Kita, Wang); Department of Pathology and Laboratory Medicine, University of California, Los Angeles, Los Angeles (Bhuta).
1. Smith SC, Patel RM, Lucas DR, McHugh JB. Sinonasal lobular capillary hemangioma: a clinicopathologic study of 34 cases characterizing potential for local recurrence. Head Neck Pathol. 2013;7(2):129-134.
Corresponding Author: Marilene B. Wang, MD, Department of Head and Neck Surgery, University of California, Los Angeles, 200 Medical Plaza, Ste 550, Los Angeles, CA 90095-6959 ([email protected]).
2. Choudhary S, MacKinnon CA, Morrissey GP, Tan ST. A case of giant nasal pyogenic granuloma gravidarum. J Craniofac Surg. 2005;16(2):319-321.
Section Editor: Edward B. Stelow, MD. Published Online: January 22, 2015. doi:10.1001/jamaoto.2014.3539. Conflict of Interest Disclosures: None reported.
388
mobile, nonpulsatile polypoid mass. Lesions affect individuals of all ages and have no preference with regard sex.6 Sixty-seven percent of lesions involve the septum; 18%, the vestibule; 12%, the turbinate; and 3%, the ethmoid sinus.1 Imaging shows a soft-tissue enhancing mass. Bony destruction is rare.7 Contrast-enhanced computed tomography shows an enhancing mass with a surrounding border of isoattenuation or hypoattenuation. Magnetic resonance imaging shows hypointensity on T1-weighted imaging, hyperintensity on T2-weighted imaging, and enhancement with gadolinium. Imaging has been found to be useful in assessing the vascular nature of these lesions and in assessing the extent of involvement, although often unnecessary for welllocalized lesions of small size.8 Treatment typically involves embolization and excision. Paranasal endoscopic techniques are most common and are often accompanied by further treatment, such as electrocautery, silver nitrate, or absorbable hemostatic agents.1 While small lesions are amenable to endoscopic removal, larger lesions have typically necessitated open craniofacial techniques, with bleeding being the main deterrent. 7 This case demonstrates that even large lesions with intradural extension can be completely resected via endoscopic techniques. While a review 4 performed in 2006 found no recurrence among the 40 cases included, a 2013 review1 of 34 cases found recurrence in up to 42% of individuals with nasal lobular capillary hemangioma. This review found that lesions recur an average of 5.7 months later. Whether this represents a failure to excise the lesion completely or an increase in spontaneous cellular changes among susceptible hosts is unclear. In addition, within adolescents and gravid women, lesions may be observed to gradually regress over a period of weeks without intervention.5 Lobular capillary hemangiomas of the nasal cavity are rare and may be detected later than those of the oral cavity. They should be considered in the presence of any enlarging vascular lesion within the nasal cavities.
3. Truss L, Dobin SM, Donner LR. Deletion (21)(q21.2q22.12) as a sole clonal cytogenetic abnormality in a lobular capillary hemangioma of the nasal cavity. Cancer Genet Cytogenet. 2006;170 (1):69-70.
5. Zarrinneshan AA, Zapanta PE, Wall SJ. Nasal pyogenic granuloma. Otolaryngol Head Neck Surg. 2007;136(1):130-131. 6. Patil P, Singla S, Mane R, Jagdeesh KS. Nasal lobular capillary hemangioma. J Clin Imaging Sci. 2013;3:40. 7. Lombardi D, Galtelli C, Khrais T, Morassi ML, Nicolai P. Giant hypervascular lesion of the sinonasal tract invading the anterior skull base and orbit: a puzzling case. Ann Otol Rhinol Laryngol. 2008;117(9):653-658. 8. Lee DG, Lee SK, Chang HW, et al. CT features of lobular capillary hemangioma of the nasal cavity. AJNR Am J Neuroradiol. 2010;31(4):749-754.
4. Puxeddu R, Berlucchi M, Ledda GP, Parodo G, Farina D, Nicolai P. Lobular capillary hemangioma of the nasal cavity: a retrospective study on 40 patients. Am J Rhinol. 2006;20(4):480-484.
JAMA Otolaryngology–Head & Neck Surgery April 2015 Volume 141, Number 4 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Purdue University User on 05/21/2015
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