- Email: [email protected]
An unusual presentation of the sternocleidomastoid tumor of infancy
OtolaryngologyHead and Neck Surgery
RONDINELLA and TOM 8181
Volume 117 Number 6
13. Silverman K, Evans SM, Strain EC, Griffiths RR, Withdrawal syndrome after the double-blind cessation of caffeine consumption. N Engl J Med 1992;327:1109-14. 14. Hughes JR, Higgins ST, Bickel WK, et al. Caffeine self-administration, withdrawa], and adverse effects among coffee drinkers. Arch Gen Psychiatry 199t ;48:61 l-7. 15. Griffiths RR, Woodson PR Caffeine physical dependence: a review of human and laboratory animal studies. Psychopharmacology 1988;94:437-51. 16. Griffiths RR, Bigelow GE, Liebson IA. Human coffee drinking: reinforcing and physical dependence-producing effects of caffeine. I Pharmacol Exp Ther 1986;239:416-25. 17. Mendel E Die schadlichen Folgen des chronischen Kaffeemissbrauchs. Berl Klin Wochenschr 1889;26:877-80.
18. Fenne!ly M, Ga!letly DC, Purdie GI. is caffeine withdrawaJ the mechanism of postoperative headache? Anesth Analg 199t ;72: 449-53. 19. Galletty DC, Fennelly M, Whitman JG. Does caffeine withdrawal contribute to postanaesthetic morbidity? Lancet 1989;1:I 335, 20. Griffitbs RR, Evans SM, Heishman SJ, et al. Low-dose caffeine physical dependence in humans. J Pharmacol Exp Ther 1990; 255:1!23-32. 2 I. Cobbs LW. Lethargy, anxiety, and impotence in a diabetic. Hosp Pract (Off Ed) 1982;17:67-73. 22. Dreisbach RH, Pfeiffer C. Caffeine-withdrawal headact~e. J Lab Clin Med !943;28:1212-9. 23. Greden JF. Anxiety or caffeinism: a diagnostic dilemma. Am J Psychiatry 1974;i31:1089-92.
An unusual presentation of the sternocleidomastoid tumor of infancy LOUIS J. RONDINELLA, MD, and lAWRENCE W. C. TOM, MD, Philadelphia, Pennsylvania
T h e sternocleidomastoid (SCM) tumor of infancy is an uncommon form of perinatal fibromatosis that results in the formation of a hard mass within the SCM muscle and is often associated with torticollis. Its diagnosis usually rests on the history and clinical presentation. If the diagnosis is unequivocal, it is usually treated with passive and active exercises to prevent o17 treat torticollis. Because the lesion frequently resolves spontaneously, some authors have advocated close observation with no specific therapy. The SCM tumor may occasionally present in an unusual manner, creating a diagnostic challenge. If there is any doubt of the diagnosis, CT or MRI and biopsy may be performed to establish the diagnosis. 1 An unusual case of SCM tumor of infancy is presented to emphasize the occasional difficulty in establishing the diagnosis.
A chest x-ray evaluation was normal. CT of the neck with contrast demonstrated a bilobed mass within the SCM muscle (Fig. I). MRI revealed a cystic septated mass in the left posterior triangle (Fig. 2). A biopsy was performed because of the suspicion of a malignancy. The mass appeared as a tan-white, well-defined lesion within the SCM muscle and fe]t rubbery (Fig. 3). Histologlc evaluation revealed coarse bundles of fibroblasts with a plexiform appearance. No a[ypia or mitotic activity was present (Fig. 4l. The diagnosis was a variant of
CASE REPORT A 10-month-old girl presented with a 1-week history of a painless left neck mass. The mass appeared suddenly and had not grown in size since its appearance. No history of trauma or infections was reported. Physical examination revealed a female child with a 4 to 5 cm firm, nontender, bilobed neck mass within the left SCM muscle. No torticollis was present. The remainder of her examination was normal.
From the Division of Otolaryngology, The Children's Hospital of Philadelphia, and the Department of Otolaryngology-Head and Neck Surgery, The University of Pennsylvania School of Medicine. Reprint requests: Lawrence W. C, Tom, MD, Division of Otolaryngology, The Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104. Otolaryngol Head Neck Surg 1997;117:S181-SI83. Copyright © 1997 by the American Academy of OtolaryngoiogyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0 23/4/75595
Fig. 1. CT scan showing o b l o n g bilobular mass in left side of neck measuring 2 x 3.5 x 4 c m with distortion of SCM muscUe. Mass follows course of SCM muscle a n d a p p e a r s to be c o n t a i n e d by muscle sheath, No calcification is seen within mass, and fat planes are preserved.
$182 RONDINELLA and TOM
Fig, 2. MRI of head and neck showing cystic mass lesion in left posterior triangle of neck. Lesion is septated and measures 3 cm x 3 cm x 2 cm. There is medial and anterior displacement of SCM muscle. Lesion is within deep fascia. No evidence of lymphadenopathy is seen.
OtolaryngologyHead and Neck Surgery December 1997
Fig. 3. Gross lesion as white, bilobed, mucoid mass measuring 3 cm x 2 cm x 1.4 cm.
Fig. 4. Histologic examination showing coarse fibromyxoid bundles producing plexiform appearance with replacement of muscle bundles, Periphery of each muscle bundle contains best preserved muscle cells with progressive rarefaction centrally. There is mild inflammatory component consisting mainly of peripheral lymphoid aggregates. There is no atypia or mitotic activity. (Hematoxylin eosin stain; original magnification x50.)
fibromatosis. The patient has done well after surgery without recurrence or development of torticollis.
DISCUSSION The terms '°fibromatosis c01i," "SCM tumor of infancy," and "congenital torticollis" are used interchangeably in the literature 2 to describe a condition characterized by fibrosis within the SCM muscle with development of a well-defined mass in the muscle and possible torticollis.
The lesion typically presents as a firm, nontender, spindleshaped mass within the distal third of the SCM muscle in children between 2 and 4 weeks of age. The mass may increase in size for a few weeks, stabilize for 2 to 3 months, and slowly resolve by 4 to 8 months of age. Frequently, affected patients are first-born children, and there is often a history of difficult labor and delivery. The mass may be associated with torticollis. This condition results from intramuscular fibrosis with subsequent
OtolaryngologyHead and Neck Surgery
KIEFF et al. $183
Volume 117 Number 6
shortening of the SCM muscle. The torticollis is usually transient and disappears as the lesion resolves. The craniofacial deformity, plagiocephaly, may be a sequelae of the torticollis. In some children the tordcollis will reappear at a later date without the reappearance of a mass. CT or MRI is indicated if the diagnosis of SCM tumor is equivocal. The CT characteristics include a mass infiltrating the SCM muscle and has attenuation values similar to those of the uninvolved portion of the muscle. 3 Its appearance on MRI is characterized by an infiltrative growth with low signal intensity on Tl-weighted images but may show low or high signal intensity on the Tz-weighted images. 4 If a diagnosis cannot be established by imaging, a biopsy is required. The gross appearance of the lesion is a discrete, round to ovoid, firm, fibrous mass within the SCM muscle. Its borders are ill defined because of its tendency to infiltrate into the surrounding muscle. 5 Several microscopic patterns reflect progressive stages in the differentiation of fibromatosis. The immature form of the "diffuse type" is the most common type. It is characterized by a dense meshwork of reticulin fibers surrounded by mucoid material. The cells appear as immature fibroblasts. A second form is represented by mature spindle-shaped fibrobtasts arranged in bundles and fascicles associated with varying amounts of collagen. Several other forms of fibromatosis occur in children older than 5 years of age. 6 Treatment of the SCM tumor of infancy initially is non-
surgical with passive and active exercises to prevent torticollis and plagiocephaly. If the mass or tol~icollis persists beyond 1 year or craniofacial asymmetry develops, excision or an open section of the muscle is indicated. This case demonstrates unusual features of fibromatosis coll. The neck mass presented at the age of t0 months, not the typical presentation of 2 to 4 weeks. The CT demonstrated a bilobular cervical mass lacking the typical infiltrative feature of fibromatosis. MRI revealed a septated cystic mass without muscle infiltration, whereas the lesion usually demonstrates nonseptation and muscle infiltration. The histopathologic makeup included an unusual plexiform appearance, and the mass lacked the dense hytinized cotlagen that is found ir~ most cases of the SCM tumor of infancy. REFERENCES I. Tom LWC, Handler SD. The sternocleidomastoid tumor of infancy. Int J Pediatr Otorhinolaryngol 1987; 13:245-55. 2. Thomsen JR, Koltal PJ. Sternomastoid tumor of infancy. Ann Otoi Rhinot Laryngol I989;98:955-9. 3. Norton KI, Shugar JM. Sternocleidomastoid tumor of infancy: CT manifestations. J Comput Assist Tomog 1991;15:158-9. 4. Liu R Tgomer R MR1 of fibromatosis: with pathologic correlation. Pediatr Radiol 1992;22:587-9. 5. el-Sayed Y. Fibromatosis of the head and neck. J Laryngoi Otol 1992; 106:459-62. 6. -Weiss SW, Enzinger FM. Fibrous proliferations of infancy and childhood. In: Soft tissue tumors. 2nd ed. C.V. Mosby Co.; 1988. p. 187.
Isolated neurosarcoidosis presenting as anosmia and
visual changes DAVID A. KIEFF,MD, HOWARD BOEY, MD, PAMELA W. SCHAEFER, MD, MAX GOODMAN, MD, and MICHAEL P. JOSEPH, MD, Boston, Massachusetts, and New Haven, Connecticut
Neurosarcoidosis (NS) is a very rare entity. Isolated cases have been reported in the literature. Intracranial sarcoidosis may present with protean manifestations. It is important to be aware of this entity, because it may easily be mistaken for other diagnoses, resulting in ineffective and inappropriate treatment plans. We describe one such case that presented in a very unusual manner with anosmia and visual changes.
From the Department of OtolaryngoIogy-Head and Neck Surgery (Drs. Kieff and Joseph) and the Department of ENT Pathology (Dr. Goodman), Massachusetts Eye and Ear Infirmary, and the Department of Radiology (Drs. Schaefer and Goodman), the Division of Neuroradiology, Massachusetts General Hospital. Reprint requests: David A. Kieff, MD, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear, 243 Charles St., Boston, MA 02114. Otolaryngol Head Neck Surg 1997;117:S183-S186. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0
2314/75594
Moreover, the radiographic and clinical evidence were most suggestive of meningioma.
CASE REPORT A 51-year-old white man arrived at the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital with a 6-year history of anosmia and 6 weeks of visual difficulty. He specifically reported difficulty tracking entries across a page during his job as an accountant. The patient's medical history was noncontributory except for a 7-year history of "mild sinusitis" treated medically with oral antibiotics during the winter. A sinus series obtained in the past year was reportedly normal. He denied any trauma, diabetes, substance abuse, HIV risk factors, or toxin exposure. He was without allergies and took no medications. He smoked periodicaIly and drank up to two whiskies per night. The patient was initially evaluated by the neurology and otolaryngology services. Examination revealed a middle-aged man in no distress. His head and neck examination was
RONDINELLA and TOM 8181
Volume 117 Number 6
13. Silverman K, Evans SM, Strain EC, Griffiths RR, Withdrawal syndrome after the double-blind cessation of caffeine consumption. N Engl J Med 1992;327:1109-14. 14. Hughes JR, Higgins ST, Bickel WK, et al. Caffeine self-administration, withdrawa], and adverse effects among coffee drinkers. Arch Gen Psychiatry 199t ;48:61 l-7. 15. Griffiths RR, Woodson PR Caffeine physical dependence: a review of human and laboratory animal studies. Psychopharmacology 1988;94:437-51. 16. Griffiths RR, Bigelow GE, Liebson IA. Human coffee drinking: reinforcing and physical dependence-producing effects of caffeine. I Pharmacol Exp Ther 1986;239:416-25. 17. Mendel E Die schadlichen Folgen des chronischen Kaffeemissbrauchs. Berl Klin Wochenschr 1889;26:877-80.
18. Fenne!ly M, Ga!letly DC, Purdie GI. is caffeine withdrawaJ the mechanism of postoperative headache? Anesth Analg 199t ;72: 449-53. 19. Galletty DC, Fennelly M, Whitman JG. Does caffeine withdrawal contribute to postanaesthetic morbidity? Lancet 1989;1:I 335, 20. Griffitbs RR, Evans SM, Heishman SJ, et al. Low-dose caffeine physical dependence in humans. J Pharmacol Exp Ther 1990; 255:1!23-32. 2 I. Cobbs LW. Lethargy, anxiety, and impotence in a diabetic. Hosp Pract (Off Ed) 1982;17:67-73. 22. Dreisbach RH, Pfeiffer C. Caffeine-withdrawal headact~e. J Lab Clin Med !943;28:1212-9. 23. Greden JF. Anxiety or caffeinism: a diagnostic dilemma. Am J Psychiatry 1974;i31:1089-92.
An unusual presentation of the sternocleidomastoid tumor of infancy LOUIS J. RONDINELLA, MD, and lAWRENCE W. C. TOM, MD, Philadelphia, Pennsylvania
T h e sternocleidomastoid (SCM) tumor of infancy is an uncommon form of perinatal fibromatosis that results in the formation of a hard mass within the SCM muscle and is often associated with torticollis. Its diagnosis usually rests on the history and clinical presentation. If the diagnosis is unequivocal, it is usually treated with passive and active exercises to prevent o17 treat torticollis. Because the lesion frequently resolves spontaneously, some authors have advocated close observation with no specific therapy. The SCM tumor may occasionally present in an unusual manner, creating a diagnostic challenge. If there is any doubt of the diagnosis, CT or MRI and biopsy may be performed to establish the diagnosis. 1 An unusual case of SCM tumor of infancy is presented to emphasize the occasional difficulty in establishing the diagnosis.
A chest x-ray evaluation was normal. CT of the neck with contrast demonstrated a bilobed mass within the SCM muscle (Fig. I). MRI revealed a cystic septated mass in the left posterior triangle (Fig. 2). A biopsy was performed because of the suspicion of a malignancy. The mass appeared as a tan-white, well-defined lesion within the SCM muscle and fe]t rubbery (Fig. 3). Histologlc evaluation revealed coarse bundles of fibroblasts with a plexiform appearance. No a[ypia or mitotic activity was present (Fig. 4l. The diagnosis was a variant of
CASE REPORT A 10-month-old girl presented with a 1-week history of a painless left neck mass. The mass appeared suddenly and had not grown in size since its appearance. No history of trauma or infections was reported. Physical examination revealed a female child with a 4 to 5 cm firm, nontender, bilobed neck mass within the left SCM muscle. No torticollis was present. The remainder of her examination was normal.
From the Division of Otolaryngology, The Children's Hospital of Philadelphia, and the Department of Otolaryngology-Head and Neck Surgery, The University of Pennsylvania School of Medicine. Reprint requests: Lawrence W. C, Tom, MD, Division of Otolaryngology, The Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104. Otolaryngol Head Neck Surg 1997;117:S181-SI83. Copyright © 1997 by the American Academy of OtolaryngoiogyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0 23/4/75595
Fig. 1. CT scan showing o b l o n g bilobular mass in left side of neck measuring 2 x 3.5 x 4 c m with distortion of SCM muscUe. Mass follows course of SCM muscle a n d a p p e a r s to be c o n t a i n e d by muscle sheath, No calcification is seen within mass, and fat planes are preserved.
$182 RONDINELLA and TOM
Fig, 2. MRI of head and neck showing cystic mass lesion in left posterior triangle of neck. Lesion is septated and measures 3 cm x 3 cm x 2 cm. There is medial and anterior displacement of SCM muscle. Lesion is within deep fascia. No evidence of lymphadenopathy is seen.
OtolaryngologyHead and Neck Surgery December 1997
Fig. 3. Gross lesion as white, bilobed, mucoid mass measuring 3 cm x 2 cm x 1.4 cm.
Fig. 4. Histologic examination showing coarse fibromyxoid bundles producing plexiform appearance with replacement of muscle bundles, Periphery of each muscle bundle contains best preserved muscle cells with progressive rarefaction centrally. There is mild inflammatory component consisting mainly of peripheral lymphoid aggregates. There is no atypia or mitotic activity. (Hematoxylin eosin stain; original magnification x50.)
fibromatosis. The patient has done well after surgery without recurrence or development of torticollis.
DISCUSSION The terms '°fibromatosis c01i," "SCM tumor of infancy," and "congenital torticollis" are used interchangeably in the literature 2 to describe a condition characterized by fibrosis within the SCM muscle with development of a well-defined mass in the muscle and possible torticollis.
The lesion typically presents as a firm, nontender, spindleshaped mass within the distal third of the SCM muscle in children between 2 and 4 weeks of age. The mass may increase in size for a few weeks, stabilize for 2 to 3 months, and slowly resolve by 4 to 8 months of age. Frequently, affected patients are first-born children, and there is often a history of difficult labor and delivery. The mass may be associated with torticollis. This condition results from intramuscular fibrosis with subsequent
OtolaryngologyHead and Neck Surgery
KIEFF et al. $183
Volume 117 Number 6
shortening of the SCM muscle. The torticollis is usually transient and disappears as the lesion resolves. The craniofacial deformity, plagiocephaly, may be a sequelae of the torticollis. In some children the tordcollis will reappear at a later date without the reappearance of a mass. CT or MRI is indicated if the diagnosis of SCM tumor is equivocal. The CT characteristics include a mass infiltrating the SCM muscle and has attenuation values similar to those of the uninvolved portion of the muscle. 3 Its appearance on MRI is characterized by an infiltrative growth with low signal intensity on Tl-weighted images but may show low or high signal intensity on the Tz-weighted images. 4 If a diagnosis cannot be established by imaging, a biopsy is required. The gross appearance of the lesion is a discrete, round to ovoid, firm, fibrous mass within the SCM muscle. Its borders are ill defined because of its tendency to infiltrate into the surrounding muscle. 5 Several microscopic patterns reflect progressive stages in the differentiation of fibromatosis. The immature form of the "diffuse type" is the most common type. It is characterized by a dense meshwork of reticulin fibers surrounded by mucoid material. The cells appear as immature fibroblasts. A second form is represented by mature spindle-shaped fibrobtasts arranged in bundles and fascicles associated with varying amounts of collagen. Several other forms of fibromatosis occur in children older than 5 years of age. 6 Treatment of the SCM tumor of infancy initially is non-
surgical with passive and active exercises to prevent torticollis and plagiocephaly. If the mass or tol~icollis persists beyond 1 year or craniofacial asymmetry develops, excision or an open section of the muscle is indicated. This case demonstrates unusual features of fibromatosis coll. The neck mass presented at the age of t0 months, not the typical presentation of 2 to 4 weeks. The CT demonstrated a bilobular cervical mass lacking the typical infiltrative feature of fibromatosis. MRI revealed a septated cystic mass without muscle infiltration, whereas the lesion usually demonstrates nonseptation and muscle infiltration. The histopathologic makeup included an unusual plexiform appearance, and the mass lacked the dense hytinized cotlagen that is found ir~ most cases of the SCM tumor of infancy. REFERENCES I. Tom LWC, Handler SD. The sternocleidomastoid tumor of infancy. Int J Pediatr Otorhinolaryngol 1987; 13:245-55. 2. Thomsen JR, Koltal PJ. Sternomastoid tumor of infancy. Ann Otoi Rhinot Laryngol I989;98:955-9. 3. Norton KI, Shugar JM. Sternocleidomastoid tumor of infancy: CT manifestations. J Comput Assist Tomog 1991;15:158-9. 4. Liu R Tgomer R MR1 of fibromatosis: with pathologic correlation. Pediatr Radiol 1992;22:587-9. 5. el-Sayed Y. Fibromatosis of the head and neck. J Laryngoi Otol 1992; 106:459-62. 6. -Weiss SW, Enzinger FM. Fibrous proliferations of infancy and childhood. In: Soft tissue tumors. 2nd ed. C.V. Mosby Co.; 1988. p. 187.
Isolated neurosarcoidosis presenting as anosmia and
visual changes DAVID A. KIEFF,MD, HOWARD BOEY, MD, PAMELA W. SCHAEFER, MD, MAX GOODMAN, MD, and MICHAEL P. JOSEPH, MD, Boston, Massachusetts, and New Haven, Connecticut
Neurosarcoidosis (NS) is a very rare entity. Isolated cases have been reported in the literature. Intracranial sarcoidosis may present with protean manifestations. It is important to be aware of this entity, because it may easily be mistaken for other diagnoses, resulting in ineffective and inappropriate treatment plans. We describe one such case that presented in a very unusual manner with anosmia and visual changes.
From the Department of OtolaryngoIogy-Head and Neck Surgery (Drs. Kieff and Joseph) and the Department of ENT Pathology (Dr. Goodman), Massachusetts Eye and Ear Infirmary, and the Department of Radiology (Drs. Schaefer and Goodman), the Division of Neuroradiology, Massachusetts General Hospital. Reprint requests: David A. Kieff, MD, Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear, 243 Charles St., Boston, MA 02114. Otolaryngol Head Neck Surg 1997;117:S183-S186. Copyright © 1997 by the American Academy of OtolaryngologyHead and Neck Surgery Foundation, Inc. 0194-5998/97/$5.00 + 0
2314/75594
Moreover, the radiographic and clinical evidence were most suggestive of meningioma.
CASE REPORT A 51-year-old white man arrived at the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital with a 6-year history of anosmia and 6 weeks of visual difficulty. He specifically reported difficulty tracking entries across a page during his job as an accountant. The patient's medical history was noncontributory except for a 7-year history of "mild sinusitis" treated medically with oral antibiotics during the winter. A sinus series obtained in the past year was reportedly normal. He denied any trauma, diabetes, substance abuse, HIV risk factors, or toxin exposure. He was without allergies and took no medications. He smoked periodicaIly and drank up to two whiskies per night. The patient was initially evaluated by the neurology and otolaryngology services. Examination revealed a middle-aged man in no distress. His head and neck examination was