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Intramuscular cavernous hemangioma of the sternocleidomastoid muscle: An unusual neck mass
Otolaryngology–Head and Neck Surgery (2007) 137, 682-683
CASE REPORT
Intramuscular cavernous hemangioma of the sternocleidomastoid muscle: An unusual neck mass Emanuele Ferri, MD, Ida Pavon, MD, and Enrico Armato, MD, Dolo (Venice), Italy
T
he first report of a case of intramuscular hemangioma is attributed to Liston in 1843, which called this entity an “erectile tumor.” Intramuscular hemangiomas (IH) are rare, benign vascular neoplasms frequently arising within the skeletal muscle of the trunk and extremities. These account for less than 1% of all hemangiomas, and head and neck involvement is considered unusual, occurring in this region only in 15% of IH. IH usually present in the second and third decades of life, with a slight male preponderance. The masseter and the trapezius muscles represent the most common site of involvement in head and neck areas. Nevertheless, fewer than 10 cases of IH of the sternocleidomastoid have been reported in the literature.1-5 A 32-year-old woman presented with a 6-month history of a single, asymptomatic, palpable mass of the right neck that recently increased in size. No history of cervical trauma prior to the appearance of the swelling was reported. Clinically, the mass was localized to the right neck centered about the middle portion of the sternocleidomastoid muscle. It was mobile on the deep planes, tender, and nonpulsatile. CT revealed a 2.5 ⫻ 1 cm, solid, ovoidal, dyshomogeneous mass within the sternocleidomastoid muscle, with peripheral calcifications. Gadolinium-enhanced MRI showed a diffusely enhancing, well-defined soft tissue lesion that demonstrated a low signal on the T1-weighted and a typical high signal on the T2-weighted MRI (Fig 1). Angiography showed no obvious feeder vessels. Echo-color Doppler showed rare arterial spots with absent venous component. The remarkable arterial resistance of the lesion was due to a probable venous thrombosis. The patient underwent transcervical surgical resection of the mass without complications. The vascular mass, predominantly consisting of venous vessels (“cavernous veins”) with partial thrombosis, was completely removed, preserving most of the sternocleidomastoid muscle and the spinal nerve. Postoperative course was uneventful and the patient was discharged early. The surgical specimen showed medium-sized and thin-walled vessels that were lined with a flattened endothelial layer and surrounded by a poorly developed and variably evident smooth-muscle sheath. The final histo-
Figure 1 Coronal gadolinium-enhanced MRI of the head and neck showed a diffusely enhancing, well-defined soft tissue lesion, measuring 2.5 ⫻ 1 cm, centered about the middle to upper portion of the posterior edge of the sternocleidomastoid muscle, with typical, marked hyperintensity on T2-weighted images.
logic diagnosis was “cavernous intramuscular hemangioma” (Fig 2). The patient has been on regular follow-up for the last six months and shows no evidence of a recurrence.
DISCUSSION A classification of IH in three categories has been proposed, based on vessel size and histologic features: cavernous (large vessels, ⬎140 m), capillary (small vessels, ⬍140 m), and mixed. Fifty percent of all IH and 68% in head and neck areas are of the capillary type. The cavernous type is less frequent than the capillary type. The cavernous IH are
Received February 2, 2007; revised April 3, 2007; accepted April 5, 2007.
0194-5998/$32.00 © 2007 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2007.04.007
Ferri et al
Intramuscular cavernous hemangioma . . .
Figure 2 Intramuscular cavernous hemangioma of the sternocleidomastoid muscle. Histologic examination showed multiple medium- and large-sized vascular spaces lined by endothelial cells admixed with striated muscle fibers, adipose cells, and fibrous tissue. (Ematoxylin and Eosin, ⫻10.)
characteristically composed of thin-walled vessels with large lumens. The endothelial cells have small nuclei, are rarely multinucleate, and show a low mitotic activity. Adipose and fibrous tissue are frequently incorporated in these tumors. Islands of bone and foci of soft tissue calcification have been reported. In contrast to capillary lesions, intravascular thrombi are much more common.1 The clinical presentation of IH is usually a palpable, localized mass with a rubbery consistency and distinct edges. Symptoms are related to mass location. Pain, when present, is secondary to neural compression. Pulsations and other findings associated with arteriovenous vascular malformations, such as bruits and thrills, are typically absent.2 The diagnosis of IH can be difficult due to its rarity and nonspecific clinical presentation. Plain radiography is commonly not diagnostic. Fine-needle aspiration biopsy of the lesion is often inconclusive due to the presence of an excessively bloody specimen. The biopsy is rarely indicated and may be dangerous. On CT with enhancement the IH typically was poorly defined, suggesting a vascular mass with the muscle, but does not allow precise differentiation between the lesion and muscle or surrounding tissues. Echocolor Doppler can reveal the vascular nature of the lesion. The MRI is very helpful, providing better than CT soft tissue delineation and spatial involvement of the lesion. MRI can accurately determine the extent of the lesion and the finding of serpentine high-volume flow voids surrounded by nonvascular soft tissue (fat, fibrous and myxoid tissue, smooth muscle, thrombus, bone) is characteristic of hemangiomas. Cavernous IH are larger than capillary IH and usually contain more nonvascular tissue. In fact, the adipose portions of these may be indistinguishable from a lipoma. Typical MRI findings in IH are hyperintense T2
683 signal. The marked hyperintensity on T2-weighted images is related to the increased free water present within the stagnant blood found in larger vessels of the neoplasm. On T1-weighted images, the IH is isointense to the skeletal muscle and has areas of increased signal approximating that of subcutaneous fat. Angiography is less performed due to its invasive nature; it is very useful in detecting feeding arteries in large IH but may fail to do so in small tumors. Nevertheless, a preoperative angiographic evaluation can identify high-flow arterial feeders with embolization and reduction of perioperative risk of hemorrhage.3-5 The differential diagnosis can be difficult and should be made with other lesions such as lymphadenopathy, salivary gland and muscular neoplasms, lymphangiomas, and thyroid lesions. In our case CT, MRI, and echo-color Doppler were fundamental for the correct diagnosis being strongly suggestive for IH. Spontaneous regression or malignant transformation generally do not occur. Surgical excision is the treatment of choice for all reported cases of sternocleidomastoid IH. Steroids, chemotherapy, and radiotherapy are not effective. In the literature are discussed other treatments, such as the sclerotherapy with foam injection, laser therapy, and angiographic embolization. The recurrence is generally caused by an incomplete excision.2,5
AUTHOR INFORMATION From the Otorhinolaryngology Unit (Drs Ferri and Armato) and Surgical Pathology Unit (Dr Pavon), Hospital of Dolo. Corresponding author: Dr Emanuele Ferri, Otorhinolaryngology Unit– Surgical Department, ULSS 13—Hospital of Dolo, Riviera XXIX Aprile, 2, 30031 Dolo, Venice, Italy. E-mail address: [email protected].
AUTHOR CONTRIBUTIONS Emanuele Ferri, writer, clinical and therapeutical analysis; Ida Pavon, histopathological analysis; Enrico Armato, writer, clinical and therapeutical analysis.
REFERENCES 1. Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8 –22. 2. Lee JK, Lim SC. Intramuscular hemangiomas of the mylohyoid and sternocleidomastoid muscle. Auris Nasus Larynx 2005;32:323–7. 3. Wolf GT, Daniel F, Krause CJ, et al. Intramuscular hemangioma of the head and neck. Laryngoscope 1985;95:210 –3. 4. Chaudhary N, Jain A, Gudwani S, et al. Intramuscular haemangioma of head and neck region. J Laryngol Otol 1998;112:1199 –201. 5. Giudice M, Piazza C, Bolzoni A, et al. Head and neck intramuscular haemangioma: report of two cases with unusual localization. Eur Arch Otorhinolaryngol 2003;260:498 –501.
CASE REPORT
Intramuscular cavernous hemangioma of the sternocleidomastoid muscle: An unusual neck mass Emanuele Ferri, MD, Ida Pavon, MD, and Enrico Armato, MD, Dolo (Venice), Italy
T
he first report of a case of intramuscular hemangioma is attributed to Liston in 1843, which called this entity an “erectile tumor.” Intramuscular hemangiomas (IH) are rare, benign vascular neoplasms frequently arising within the skeletal muscle of the trunk and extremities. These account for less than 1% of all hemangiomas, and head and neck involvement is considered unusual, occurring in this region only in 15% of IH. IH usually present in the second and third decades of life, with a slight male preponderance. The masseter and the trapezius muscles represent the most common site of involvement in head and neck areas. Nevertheless, fewer than 10 cases of IH of the sternocleidomastoid have been reported in the literature.1-5 A 32-year-old woman presented with a 6-month history of a single, asymptomatic, palpable mass of the right neck that recently increased in size. No history of cervical trauma prior to the appearance of the swelling was reported. Clinically, the mass was localized to the right neck centered about the middle portion of the sternocleidomastoid muscle. It was mobile on the deep planes, tender, and nonpulsatile. CT revealed a 2.5 ⫻ 1 cm, solid, ovoidal, dyshomogeneous mass within the sternocleidomastoid muscle, with peripheral calcifications. Gadolinium-enhanced MRI showed a diffusely enhancing, well-defined soft tissue lesion that demonstrated a low signal on the T1-weighted and a typical high signal on the T2-weighted MRI (Fig 1). Angiography showed no obvious feeder vessels. Echo-color Doppler showed rare arterial spots with absent venous component. The remarkable arterial resistance of the lesion was due to a probable venous thrombosis. The patient underwent transcervical surgical resection of the mass without complications. The vascular mass, predominantly consisting of venous vessels (“cavernous veins”) with partial thrombosis, was completely removed, preserving most of the sternocleidomastoid muscle and the spinal nerve. Postoperative course was uneventful and the patient was discharged early. The surgical specimen showed medium-sized and thin-walled vessels that were lined with a flattened endothelial layer and surrounded by a poorly developed and variably evident smooth-muscle sheath. The final histo-
Figure 1 Coronal gadolinium-enhanced MRI of the head and neck showed a diffusely enhancing, well-defined soft tissue lesion, measuring 2.5 ⫻ 1 cm, centered about the middle to upper portion of the posterior edge of the sternocleidomastoid muscle, with typical, marked hyperintensity on T2-weighted images.
logic diagnosis was “cavernous intramuscular hemangioma” (Fig 2). The patient has been on regular follow-up for the last six months and shows no evidence of a recurrence.
DISCUSSION A classification of IH in three categories has been proposed, based on vessel size and histologic features: cavernous (large vessels, ⬎140 m), capillary (small vessels, ⬍140 m), and mixed. Fifty percent of all IH and 68% in head and neck areas are of the capillary type. The cavernous type is less frequent than the capillary type. The cavernous IH are
Received February 2, 2007; revised April 3, 2007; accepted April 5, 2007.
0194-5998/$32.00 © 2007 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2007.04.007
Ferri et al
Intramuscular cavernous hemangioma . . .
Figure 2 Intramuscular cavernous hemangioma of the sternocleidomastoid muscle. Histologic examination showed multiple medium- and large-sized vascular spaces lined by endothelial cells admixed with striated muscle fibers, adipose cells, and fibrous tissue. (Ematoxylin and Eosin, ⫻10.)
characteristically composed of thin-walled vessels with large lumens. The endothelial cells have small nuclei, are rarely multinucleate, and show a low mitotic activity. Adipose and fibrous tissue are frequently incorporated in these tumors. Islands of bone and foci of soft tissue calcification have been reported. In contrast to capillary lesions, intravascular thrombi are much more common.1 The clinical presentation of IH is usually a palpable, localized mass with a rubbery consistency and distinct edges. Symptoms are related to mass location. Pain, when present, is secondary to neural compression. Pulsations and other findings associated with arteriovenous vascular malformations, such as bruits and thrills, are typically absent.2 The diagnosis of IH can be difficult due to its rarity and nonspecific clinical presentation. Plain radiography is commonly not diagnostic. Fine-needle aspiration biopsy of the lesion is often inconclusive due to the presence of an excessively bloody specimen. The biopsy is rarely indicated and may be dangerous. On CT with enhancement the IH typically was poorly defined, suggesting a vascular mass with the muscle, but does not allow precise differentiation between the lesion and muscle or surrounding tissues. Echocolor Doppler can reveal the vascular nature of the lesion. The MRI is very helpful, providing better than CT soft tissue delineation and spatial involvement of the lesion. MRI can accurately determine the extent of the lesion and the finding of serpentine high-volume flow voids surrounded by nonvascular soft tissue (fat, fibrous and myxoid tissue, smooth muscle, thrombus, bone) is characteristic of hemangiomas. Cavernous IH are larger than capillary IH and usually contain more nonvascular tissue. In fact, the adipose portions of these may be indistinguishable from a lipoma. Typical MRI findings in IH are hyperintense T2
683 signal. The marked hyperintensity on T2-weighted images is related to the increased free water present within the stagnant blood found in larger vessels of the neoplasm. On T1-weighted images, the IH is isointense to the skeletal muscle and has areas of increased signal approximating that of subcutaneous fat. Angiography is less performed due to its invasive nature; it is very useful in detecting feeding arteries in large IH but may fail to do so in small tumors. Nevertheless, a preoperative angiographic evaluation can identify high-flow arterial feeders with embolization and reduction of perioperative risk of hemorrhage.3-5 The differential diagnosis can be difficult and should be made with other lesions such as lymphadenopathy, salivary gland and muscular neoplasms, lymphangiomas, and thyroid lesions. In our case CT, MRI, and echo-color Doppler were fundamental for the correct diagnosis being strongly suggestive for IH. Spontaneous regression or malignant transformation generally do not occur. Surgical excision is the treatment of choice for all reported cases of sternocleidomastoid IH. Steroids, chemotherapy, and radiotherapy are not effective. In the literature are discussed other treatments, such as the sclerotherapy with foam injection, laser therapy, and angiographic embolization. The recurrence is generally caused by an incomplete excision.2,5
AUTHOR INFORMATION From the Otorhinolaryngology Unit (Drs Ferri and Armato) and Surgical Pathology Unit (Dr Pavon), Hospital of Dolo. Corresponding author: Dr Emanuele Ferri, Otorhinolaryngology Unit– Surgical Department, ULSS 13—Hospital of Dolo, Riviera XXIX Aprile, 2, 30031 Dolo, Venice, Italy. E-mail address: [email protected].
AUTHOR CONTRIBUTIONS Emanuele Ferri, writer, clinical and therapeutical analysis; Ida Pavon, histopathological analysis; Enrico Armato, writer, clinical and therapeutical analysis.
REFERENCES 1. Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8 –22. 2. Lee JK, Lim SC. Intramuscular hemangiomas of the mylohyoid and sternocleidomastoid muscle. Auris Nasus Larynx 2005;32:323–7. 3. Wolf GT, Daniel F, Krause CJ, et al. Intramuscular hemangioma of the head and neck. Laryngoscope 1985;95:210 –3. 4. Chaudhary N, Jain A, Gudwani S, et al. Intramuscular haemangioma of head and neck region. J Laryngol Otol 1998;112:1199 –201. 5. Giudice M, Piazza C, Bolzoni A, et al. Head and neck intramuscular haemangioma: report of two cases with unusual localization. Eur Arch Otorhinolaryngol 2003;260:498 –501.