ACTINOMYCOTIC GRANULOMA MASQUERADING A S AN INFRAORBITAL NERVE NEOPLASM Gady Har-El, MD, David A. Prager, MD, Fidelina De Soto La Paix, MD, and Michael Nash, MD
Actinomycotic infections of the head and neck region are rare and frequently present a confusing clinical picture. We document a case of actinomycosis that simulated a neoplasm of the infraorbital nerve, both clinically and surgically. To the best of our knowledge, this is the first report of actinomycosis involving this nerve. Correct diagnosis of actinomycotic infection in the head and neck relies most heavily on clinical suspicion. It is, therefore, imperative for the head and neck surgeon to be familiar with the etiology of this infection and its various modes of presentation as well as its appropriate management. HEAD & NECK 12~261-263,1990
Actinomycosis is a chronic, suppurative infection caused primarily by the gram positive bacterium Actinomyces isrueli. The organism is present universally in the human mouth. It is, therefore, not surprising that the majority of infections are of the cervicofacial form. Minor oral trauma is believed to be responsible for the majority of cases, as this allows the organism to gain access t o the subcutaneous tissues.
From the Department of Otolaryngology (Drs. Har-El, Prager, and Nash). SUNY-Health Science Center at Brooklyn, and Department of Pathology (Dr. De Soto La Paix), Brooklyn Veteran’s Administration Medical Center, Brooklyn, New York. Address reprint requests to Dr. David A. Prager. The Long Island College Hospital, Department of Otolaryngology, 340 Henry Street, Brooklyn, NY 11201. Accepted for publication December 28, 1989 CCC 0148-6403/90/030261-03 $04.00 0 1990 John Wiley & Sons, Inc.
Actinomycotic Granuloma
Actinomycosis is now a rare diagnosis in the United States, due to both its extreme sensitivity to most antimicrobial medications, and to the failure of most clinicians to consider the diagnosis early in its presentation.l9’ This disease has been called a “chameleon”of head and neck pathology because of its ability to mimic various other disorders and also because of its lack of distinguishing clinical features in the initial ~ o u r s e .At ~ ’presentation, ~ it may easily be mistaken for an abscess or a benign or malignant neoplasm.2 CASE REPORT
A 71-year-old hispanic man presented to our service with a 4-week history of progressive swelling in the subcutaneous tissue overlying the right maxilla. There was no history of recent oral trauma or dental work. The patient complained of a mild hypesthesia over the right side of the face. Physical examination revealed a slightly visible, nonerythematous swelling over the right side of the face, extending a few millimeters onto the right alar groove. Palpation revealed an irregular, hard, mobile mass approximately 3 cm in greatest diameter underlying the intact normal facial skin. On bimanual examination, the mass was palpable through the buccal mucosa over the region of the right upper first premolar. The patient was noted to have poor dentition, HEAD & NECK
May/June 1990
261
with several carious teeth throughout the oral cavity. No intraoral or cutaneous fistulae were noted, and there was no cervical lymphadenopathy. A tumor of the maxillary sinus was suspected and a CT scan was obtained. This demonstrated an enhancing, irregular subcutaneous mass, completely anterior to the right maxillary sinus and with no evidence of bony destruction. (Fig. 1). Differential diagnosis at this point included a minor salivary gland tumor, subcutaneous skin adnexal tumor, and maxilloglobular cyst. Exploration through the gingivo-labial sulcus revealed a mass that appeared to arise from and completely surround the infra-orbital nerve (Fig. 2). The nerve was sacrificed in order to remove the entire mass. Frozen section at that time suggested a neoplasm of neural origin and questionable malignant character. Pathologic examination showed pyogenic granulation tissue with slender short tracks of focal necrosis. The purulent material contained large, dense, roughly oval-to-round bodies composed of gram positive filamentous organisms consistent with actinomyces species (Fig. 3). These were surrounded by an envelope of neutrophilic leukocytes. Higher magnification, particularly H & E stain, showed peripheral ray-like eosinophilic structures that represent the Splendore- Hoeppli phen~menon.~ Large bundles of myelinated nerves are entrapped in the pyogenic granuloma and remaining soft tissue (Fig. 4). Diagnosis: Pyogenic granuloma of soft tissue due to actinomycosis.
RGURE 1. Computerized tomographic scan demonstrating enhancing lesion completely anterior to right maxillary sinus.
262
Actinornycotic Granuloma
FIGURE 2. lntraoperative photograph showing tumor mass to completely surround the infraorbital nerve, which can be seen exiting from the foramen (arrow head).
COMMENT
In 1877, Bollinger described actinomyces as the organism responsible for the “lumpy jaw” disease noted in certain cattle. Israel, only a year later, described a similar condition in humans.6 While the name implies a fungal organism, the actinomyces are indisputably bacterial in nature.’ The organism is a normal inhabitant of the human mouth and tends to act as an “opportunistic” infection, gaining access to the host tissues through interruption of the normal mucosal barrier^.^ In almost all cases of cervicofacial actinomycosis, a careful review of the history and physical will implicate a dental port of entry. Characteristically, the early progress of the
FIGURE 3. Close up of gram stain showing the interlacing gram positive filaments of actinonycosis (x400).
HEAD & NECK
May/June 1990
FIGURE 4. Large myelinated nerve bundies entrapped in the pyogenic granulation tissue (H & E x200, original magnification).
disease manifests as a discrete, fluctuant mass. The infection spreads in a burrowing fashion without regard to normal anatomic planes. This leads to development of the frequently reported picture of a nodular, indurated mass or “pseudotumor.~’8~g As the disease progresses, draining sinus tracts may develop through the Proper diagnosis in the skin or oral early stage is extremely difficult. In reviewing 57 cases of actinomycosis, Weese reported that only four (7%) of the cases were correctly diagnosed. The most common erroneous initial clinical impression was that of a malignancy. Several factors account for this diagnostic enigma. Since actinomyces respond readily to most antibiotics, the mass may fluctuate in size and even temporarily disappear with a short course of treatment.7 Moreover, since the organism is so susceptible to many of the these medications, it
can seldom, if ever, be identified in patients who are receiving or have recently received antibiotics. Perhaps most significant, however, is that as the frequency of actinomycotic infections has decreased, it is usually neglected in one’s differential diagnosis. A presumptive diagnosis can be made based on findings of the classical sulfur granules, either grossly or microscopically, in specimens obtained from draining fistulae, needle aspirates, or biopsy specimens. Definitive diagnosis, however, is established by anaerobically culturing actinomycosis grains in brain heart agar or blood agar under appropriate conditions. Proper therapy consists of a prolonged course (usually months) of penicillin in high doses. Tetracycline is an effective alternative for allergic patients. Occasionally, surgical drainage or excision may be necessary for treatment failures. The case discussed above was unique in its presentation and surgical findings. Review of the medical literature revealed no similar cases. In 1987, Atri et al, described an actinomycotic granuloma of the trigeminal ganglion.12 It is interesting to speculate whether the lesion in our case was actually a granuloma of the nerve itself or whether the nerve was only involved by direct extension of the infection. Actinomycosis remains an elusive and challenging problem for the head and neck clinician. Once the diagnosis is confirmed, treatment is simple and highly effective. Proper diagnosis depends heavily on the physician’s familiarity with all aspects of the disease process, most notably, its ability to mimic a neoplasm. Finally, actinomycosis must be considered in the differential diagnosis of any undiagnosed mass in the face or neck.
REFERENCES
1. Lerner PI. The lumpy jaw: Cervicofacial actinomycosis. Infect Dis Clin 1988;2:203-220. 2. Bennhoff DF. Actinomycosis: Diagnostic and therapeutic considerations and a review of 32 cases. Laryngoscope 1984:94:1198- 1217. 3. Rankow RM, Abraham DM. Actinomycosis: Masquerader in the head and neck. Ann Otol Rhino1 Laryngol 1978,87:230-237. 4. Kusumi RK. Infections. In Cummings, CW: Otolaryngology head and neck surgery. 1986;2:1609-1620. 5. Johnson FB. Splendore-Hoeppli phenomenon. In Binford CH and Connor DH: Pathology of tropical and extraordinary diseases. Armed Forces Institute of Pathology, Washington, DC, 1976;681-683. 6. Everts EC. Cervicofacial actinomycosis. Arch Otolaryngo1 1970;92:468-474.
Actinomycotic Granuloma
7. Weese WC, Smith IM. A study of 57 cases of actinomycosis over a 36-year period. Arch Intern Med 1975;135:1562- 1568. 8. Sodagar R, Kohout E. Actinomycosis of the tongue as pseudotumor. Laryngoscope 1972;82:2149-2152, 9. Mandell GL, Douglas RG, Benett JE. Principles and Practice of Infectious Diseases. John Wiley, New York, 1979. 10. Holst, E, Lund, P Cervicofacial actinomycosis: A retrospective study. Int J Oral Surg 1979;8:194-198. 11. Brown J. Human actinomycosis: A study of 181 patients. Human Pathology 1973;4319-330. 12. Atri M, Robertson WD, Durity FA, Dolman CL. Actinomycotic granuloma of the trigeminal ganglion. AJNR 1987;8:167- 169.
HEAD & NECK
May/June 1990
263
Actinomycotic infections of the head and neck region are rare and frequently present a confusing clinical picture. We document a case of actinomycosis that simulated a neoplasm of the infraorbital nerve, both clinically and surgically. To the best of our knowledge, this is the first report of actinomycosis involving this nerve. Correct diagnosis of actinomycotic infection in the head and neck relies most heavily on clinical suspicion. It is, therefore, imperative for the head and neck surgeon to be familiar with the etiology of this infection and its various modes of presentation as well as its appropriate management. HEAD & NECK 12~261-263,1990
Actinomycosis is a chronic, suppurative infection caused primarily by the gram positive bacterium Actinomyces isrueli. The organism is present universally in the human mouth. It is, therefore, not surprising that the majority of infections are of the cervicofacial form. Minor oral trauma is believed to be responsible for the majority of cases, as this allows the organism to gain access t o the subcutaneous tissues.
From the Department of Otolaryngology (Drs. Har-El, Prager, and Nash). SUNY-Health Science Center at Brooklyn, and Department of Pathology (Dr. De Soto La Paix), Brooklyn Veteran’s Administration Medical Center, Brooklyn, New York. Address reprint requests to Dr. David A. Prager. The Long Island College Hospital, Department of Otolaryngology, 340 Henry Street, Brooklyn, NY 11201. Accepted for publication December 28, 1989 CCC 0148-6403/90/030261-03 $04.00 0 1990 John Wiley & Sons, Inc.
Actinomycotic Granuloma
Actinomycosis is now a rare diagnosis in the United States, due to both its extreme sensitivity to most antimicrobial medications, and to the failure of most clinicians to consider the diagnosis early in its presentation.l9’ This disease has been called a “chameleon”of head and neck pathology because of its ability to mimic various other disorders and also because of its lack of distinguishing clinical features in the initial ~ o u r s e .At ~ ’presentation, ~ it may easily be mistaken for an abscess or a benign or malignant neoplasm.2 CASE REPORT
A 71-year-old hispanic man presented to our service with a 4-week history of progressive swelling in the subcutaneous tissue overlying the right maxilla. There was no history of recent oral trauma or dental work. The patient complained of a mild hypesthesia over the right side of the face. Physical examination revealed a slightly visible, nonerythematous swelling over the right side of the face, extending a few millimeters onto the right alar groove. Palpation revealed an irregular, hard, mobile mass approximately 3 cm in greatest diameter underlying the intact normal facial skin. On bimanual examination, the mass was palpable through the buccal mucosa over the region of the right upper first premolar. The patient was noted to have poor dentition, HEAD & NECK
May/June 1990
261
with several carious teeth throughout the oral cavity. No intraoral or cutaneous fistulae were noted, and there was no cervical lymphadenopathy. A tumor of the maxillary sinus was suspected and a CT scan was obtained. This demonstrated an enhancing, irregular subcutaneous mass, completely anterior to the right maxillary sinus and with no evidence of bony destruction. (Fig. 1). Differential diagnosis at this point included a minor salivary gland tumor, subcutaneous skin adnexal tumor, and maxilloglobular cyst. Exploration through the gingivo-labial sulcus revealed a mass that appeared to arise from and completely surround the infra-orbital nerve (Fig. 2). The nerve was sacrificed in order to remove the entire mass. Frozen section at that time suggested a neoplasm of neural origin and questionable malignant character. Pathologic examination showed pyogenic granulation tissue with slender short tracks of focal necrosis. The purulent material contained large, dense, roughly oval-to-round bodies composed of gram positive filamentous organisms consistent with actinomyces species (Fig. 3). These were surrounded by an envelope of neutrophilic leukocytes. Higher magnification, particularly H & E stain, showed peripheral ray-like eosinophilic structures that represent the Splendore- Hoeppli phen~menon.~ Large bundles of myelinated nerves are entrapped in the pyogenic granuloma and remaining soft tissue (Fig. 4). Diagnosis: Pyogenic granuloma of soft tissue due to actinomycosis.
RGURE 1. Computerized tomographic scan demonstrating enhancing lesion completely anterior to right maxillary sinus.
262
Actinornycotic Granuloma
FIGURE 2. lntraoperative photograph showing tumor mass to completely surround the infraorbital nerve, which can be seen exiting from the foramen (arrow head).
COMMENT
In 1877, Bollinger described actinomyces as the organism responsible for the “lumpy jaw” disease noted in certain cattle. Israel, only a year later, described a similar condition in humans.6 While the name implies a fungal organism, the actinomyces are indisputably bacterial in nature.’ The organism is a normal inhabitant of the human mouth and tends to act as an “opportunistic” infection, gaining access to the host tissues through interruption of the normal mucosal barrier^.^ In almost all cases of cervicofacial actinomycosis, a careful review of the history and physical will implicate a dental port of entry. Characteristically, the early progress of the
FIGURE 3. Close up of gram stain showing the interlacing gram positive filaments of actinonycosis (x400).
HEAD & NECK
May/June 1990
FIGURE 4. Large myelinated nerve bundies entrapped in the pyogenic granulation tissue (H & E x200, original magnification).
disease manifests as a discrete, fluctuant mass. The infection spreads in a burrowing fashion without regard to normal anatomic planes. This leads to development of the frequently reported picture of a nodular, indurated mass or “pseudotumor.~’8~g As the disease progresses, draining sinus tracts may develop through the Proper diagnosis in the skin or oral early stage is extremely difficult. In reviewing 57 cases of actinomycosis, Weese reported that only four (7%) of the cases were correctly diagnosed. The most common erroneous initial clinical impression was that of a malignancy. Several factors account for this diagnostic enigma. Since actinomyces respond readily to most antibiotics, the mass may fluctuate in size and even temporarily disappear with a short course of treatment.7 Moreover, since the organism is so susceptible to many of the these medications, it
can seldom, if ever, be identified in patients who are receiving or have recently received antibiotics. Perhaps most significant, however, is that as the frequency of actinomycotic infections has decreased, it is usually neglected in one’s differential diagnosis. A presumptive diagnosis can be made based on findings of the classical sulfur granules, either grossly or microscopically, in specimens obtained from draining fistulae, needle aspirates, or biopsy specimens. Definitive diagnosis, however, is established by anaerobically culturing actinomycosis grains in brain heart agar or blood agar under appropriate conditions. Proper therapy consists of a prolonged course (usually months) of penicillin in high doses. Tetracycline is an effective alternative for allergic patients. Occasionally, surgical drainage or excision may be necessary for treatment failures. The case discussed above was unique in its presentation and surgical findings. Review of the medical literature revealed no similar cases. In 1987, Atri et al, described an actinomycotic granuloma of the trigeminal ganglion.12 It is interesting to speculate whether the lesion in our case was actually a granuloma of the nerve itself or whether the nerve was only involved by direct extension of the infection. Actinomycosis remains an elusive and challenging problem for the head and neck clinician. Once the diagnosis is confirmed, treatment is simple and highly effective. Proper diagnosis depends heavily on the physician’s familiarity with all aspects of the disease process, most notably, its ability to mimic a neoplasm. Finally, actinomycosis must be considered in the differential diagnosis of any undiagnosed mass in the face or neck.
REFERENCES
1. Lerner PI. The lumpy jaw: Cervicofacial actinomycosis. Infect Dis Clin 1988;2:203-220. 2. Bennhoff DF. Actinomycosis: Diagnostic and therapeutic considerations and a review of 32 cases. Laryngoscope 1984:94:1198- 1217. 3. Rankow RM, Abraham DM. Actinomycosis: Masquerader in the head and neck. Ann Otol Rhino1 Laryngol 1978,87:230-237. 4. Kusumi RK. Infections. In Cummings, CW: Otolaryngology head and neck surgery. 1986;2:1609-1620. 5. Johnson FB. Splendore-Hoeppli phenomenon. In Binford CH and Connor DH: Pathology of tropical and extraordinary diseases. Armed Forces Institute of Pathology, Washington, DC, 1976;681-683. 6. Everts EC. Cervicofacial actinomycosis. Arch Otolaryngo1 1970;92:468-474.
Actinomycotic Granuloma
7. Weese WC, Smith IM. A study of 57 cases of actinomycosis over a 36-year period. Arch Intern Med 1975;135:1562- 1568. 8. Sodagar R, Kohout E. Actinomycosis of the tongue as pseudotumor. Laryngoscope 1972;82:2149-2152, 9. Mandell GL, Douglas RG, Benett JE. Principles and Practice of Infectious Diseases. John Wiley, New York, 1979. 10. Holst, E, Lund, P Cervicofacial actinomycosis: A retrospective study. Int J Oral Surg 1979;8:194-198. 11. Brown J. Human actinomycosis: A study of 181 patients. Human Pathology 1973;4319-330. 12. Atri M, Robertson WD, Durity FA, Dolman CL. Actinomycotic granuloma of the trigeminal ganglion. AJNR 1987;8:167- 169.
HEAD & NECK
May/June 1990
263
