BACKGROUND
Lobular capillary hemangiomas (LCH) are the benign tumors of vascular origin, commonly affecting the mucous membranes and the superficial skin. They are characterized by rapid growth and friable surface and often profuse bleeding. Other uncommon and rare variants include LCH gravidum, intravenous LCH, LCH with satellite lesions, and subcutaneous LCH. Subcutaneous LCH form is rare with very few case repots. Subcutaneous LCH lacks the characteristic surface properties and present as deeper nodules, making it difficult to diagnose clinically. We present the case of a 22-year-old female presenting with a subcutaneous LCH in the supraclavicular region, mimicking a brachial plexus neurofibroma.
CASE REPORT
A 22-year-old female presented to us with a left supraclavicular mass first noticed 5 years back. The lesion gradually progressed in size and was not associated with pain. She had undergone exploration and partial resection of the lesion at another hospital 3 years back with surgery being abandoned. Immediately, after the surgery, she developed winging of left scapula suggesting an iatrogenic injury to the left long thoracic nerve. No biopsy report from the previous surgery was available. The residual swelling had gradually progressed over time.
On examination, the patient had a 5 cm × 5 cm × 4 cm swelling in the left supraclavicular region in the subcutaneous plane. It had a smooth surface, firm consistency, distinct margins and was non tender to touch. Neurological examination revealed weakness in shoulder abduction with a power of 3/5, shoulder rotation-3/5 and elbow flexion-4/5. A sensory loss of around 30%–40% was also noticed in the left upper lateral arm. Further examination also revealed a medial winging of scapula on the same side.
Imaging revealed 56 mm × 42 mm × 49 mm lobulated lesion in the left supraclavicular region with multiple flow voids involving the brachial plexus and vascularization from left subclavian artery. The lesion was isointense on T1, hyperintense on short-tau inversion recovery, and T2 imaging [Figure 1] and enhancing on contrast imaging. Nerve conduction studies were suggestive of a left suprascapular and axillary neuropathy with ongoing denervation and evidence of chronic reinnervation.
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Figure 1: MRI images of the lesion (a) Coronal T1 section (b) Axial T1 section (c) Coronal T2 section (d) Axial STIR section. STIR = Short tau inversion recovery, MRI = Magnetic resonance imaging
A surgical excision was planned. The patient was placed in the supine position with neck rotated to the right side. A 4 channel NIM (nerve integrity monitor, Medtronic Memphis, TN, USA) was planned to be used and appropriate electrodes were placed in appropriate shoulder, arm and forearm muscles. A longitudinal incision along the base of the neck was planned at the site of the previous incision and subplatysmal dissection was carried out to visualize the tumor. Tumor was encapsulated, grayish white in color and firm in consistency. Although the capsule of the tumor was adhered at the surrounding tissue a plane could be made out by gradual careful dissection, extracapsular dissection was carried out and NIM probe used at each step to delineate functional neural tissue [Figure 2]. Careful dissection was carried out and functional nerves could be identified on the medial, lateral and inferior aspects the tumor which were delineated and preserved. A gross total excision was achieved. Integrity of brachial plexus branches reconfirmed using NIM probe. Hemostasis was achieved and closure was done in layers. Postoperatively, the patient had an uneventful postoperative hospital stay and had no new deficits.
Figure 2: (a) Image showing the patient positioning for the case. (b) Image showing intraoperative image for the case showing a the tumor adhered to the axillary nerve (shown by forceps) on the lateral side. (c) Gross section and (d) Cut section of the tumor
Histopathological examination revealed well-circumscribed vascular lesion arranged in lobules, comprising of variable sized capillaries admixed with larger vessels. Hobnailing of endothelial nuclei was absent. Endothelial cells were immunopositive for with CD-31, CD-34, D2-40. These features were in line with a diagnosis of LCH, adult type [Figure 3].
Figure 3: Histopathological and IHC images (a) Hematoxylin and Eosin stain show well circumscribed vascular lesion arranged in lobules, comprising of variable sized capillaries admixed with larger vessels. IHC images showing endothelial cells were immunopositive for with (b) CD-31 (c) CD-34 and (d) D2-40. IHC = Immunohistochemistry
DISCUSSION
LCH or pyogenic granuloma is a benign pathology that is made up of capillary clusters set up in a lobular architecture with fibrous bands separating the lobules.[1] LCH is most commonly seen in children and young adults with mucosal and dermal forms being most common.[2] Some of the other forms include intravenous, satellitosis, LCH gravidum, and subcutaneous forms. Subcutaneous form is very rare, forming <5% of all cases of LCH.[3] A few case reports have reported subcutaneous LCH to occur at face, neck, abdominal wall, and extremities.[4–6] However, to the best of our knowledge, we report the first case of a subcutaneous LCH involving the brachial plexus.
The mucosal and dermal forms of LCH are often raised, friable lesions that bleed easily on touch. However, subcutaneous forms lack these characteristics and present as deep nodules with no characteristic appearance, making clinical diagnosis very challenging.[3] The lesion in the present case was isointense on T1, hyperintense on T2, and homogenously enhancing on contrast. A schwannoma which is a more common lesion at this location with similar radiological findings was kept as the presumptive diagnosis in this case. It can be extremely difficult to radiologically differentiate between the two.
Surgical excision is the mainstay of treatment in these cases. The lesion is often well encapsulated and can be removed in toto. Operating around the brachial plexus can be challenging with high rates (16%–50%) of postoperative functional deficits being reported.[7,8] Intraoperative neuromonitoring is now widely across surgical specialties in surgeries around the nerves.[9,10] The technique facilitates identification of the nerve, minimizing trauma to the nerve during dissection, and final confirmation of neural integrity at the end of surgery. Neuromonitoring has been shown to beneficial and improve the safety of resection in case of brachial plexus tumors.[11] In the present case, a safe resection preserving the brachial plexus branches was facilitated by the use of neuromonitoring.
Localized recurrence and satellite lesions have been reported have been reported with dermal and mucosal forms of LCH. Recurrence rates as high as 16% have been reported, mostly attributed to incomplete excision.[12] However, none of the few reports of subcutaneous LCH have noted any recurrence after excision. In the present case, a partial excision had been done previously with residual lesion left being which gradually progressed over time. No adjunctive radiotherapy or chemotherapy is needed for these benign lesions.[4–6]
CONCLUSION
Subcutaneous LCH involving the brachial plexus is a rare entity. It can mimic a brachial plexus schwannoma which is more common and should be kept as a differential diagnosis. Surgical excision assisted by neuromonitoring provides good outcomes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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