Sunday, December 19, 2021
Wednesday, August 19, 2020
The patient is an active 10-year-old boy who was seen today for evaluation of a vascular anomaly on his right knee that has been present since birth. It was biopsied in March 2017 and the histologic diagnosis was "lymphatic malformation consistent with a lymphangioma."
Occasionally, his knee will swell and he will get some bleeding spots. He is learning boxing and that can precipitate swelling. He never has pain. Has not had any infection. I referred him to a pediatric dermatologist 3 years ago but they were out of network and he never went.
The lesion measures 7 cm in diameter on the right knee. There are areas of dark purplish pigmentation in confluent vesicles.
Pathology: There were multiple dilated irregular thin walled
vessels lined by a single layer of bland endothelial cells.
circumscriptum. I expect there is a deep
component. The patient has no
lymphedema. Note: The new terminology for this is "Microcystic Lymphatic Malformation (Ref 4.)
Question: Should this tumor be evaluated and if so how? Would any therapy be better than active surveillance?
Alyssa Miceli, Kristen M. Stewart In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan. 2020 Aug 12. Free Full Text.
Lymphangiomas are uncommon, benign malformations of the lymphatic
system that can occur anywhere on the skin and mucous membranes. Lymphangiomas
can be categorized as deep or superficial based on the depth and size of the
abnormal lymphatic vessels or as congenital or acquired. The deep forms of
lymphangioma include two specific well defined congenital entities: cavernous
lymphangiomas and cystic hygromas. Superficial forms of lymphangioma include
lymphangioma circumscriptum and acquired lymphangioma, which is also referred
to in the literature as lymphangiectasia. Although both entities share similar
clinical and histologic features, the term lymphangioma circumscriptum infers
lymphatic channel dilation due to a congenital malformation of the lymphatic
system. Whereas, the term lymphangiectasia, or acquired lymphangioma, denotes
dilated lymphatic channels of previously normal lymphatics that have become
obstructed by an external cause.
2. The Possible Role of Color Doppler and Pulse Wave Doppler Ultrasound
in the Diagnosis of Cutaneous Lymphangioma Circumscriptum. Yang Sun, et. al. J Craniofac Surg. Jan/Feb 2020
Abstract: Cutaneous lymphangioma circumscriptum (CLC) is an uncommon congenital lymphatic malformation. Its dermoscopic features have been reported, however, if blood has infiltrated the lacunas, a pink hue overwhelms the lacunar structures, rendering dermoscopic features indistinguishable. In addition, dermoscopy cannot assess the subcutaneous extent of the lesion before surgery. The high-frequency array transducer Sonography with a high-frequency transducer is excellent in its resolution to evaluate skin lesions and is unaffected by infiltration of blood. Here, the authors report the use of ultrasound (color Doppler and pulse wave Doppler) for the diagnosis and management of CLC.An 18-year-old man presented 10 years previously with an asymptomatic 0.5 × 0.7 cm cluster of dark reddish vesicular lesions on his buttock. The lesions had recently increased in size and number. There was associated pachyderma. The lesions were hyperechoic and well defined on grayscale ultrasound and hypervascular on color Doppler ultrasound. Cluster cystics of lymphatic spaces were found throughout the whole dermis, especially in the papillary dermis. The lymphatic channels extended downwards to the reticular dermis and the superficial layer of the subcutaneous tissue. The pathological findings revealed lymphangiomas invading the subcutaneous tissue. The flat endothelial cells were partly positive for D2-40, which is a marker of lymphatic endothelial cells. Ultrasound was able to delineate the margins of the lesions. Diagnosis of CLC using preoperative color Doppler and pulse wave Doppler could reduce unnecessary repeated pre- and postoperative biopsies.
3. Lynphangioma Overview National Organization for Rare Disorders.
4. Micro- and Macrocystic Lymphatic Malformation. Lindsay McCormack, Krystal Jones, Jennifer T Huang. J Pediatr. 2020 Apr;219:275-276. Free Full Text
5. Treatment of superficial vascular anomalies with topical sirolimus: A multicenter case series. Melissa Dodds, et. al. Pediatr Dermatol. 2020 Mar;37(2):272-277.
Abstract: Background: Systemic sirolimus (rapamycin) has recently been found effective in treating complex vascular anomalies by reducing the size and associated complications. Many vascular anomalies have a cutaneous component, and thus, we sought to determine whether topical administration of sirolimus may be an effective therapy, as data on the use of topical sirolimus are limited.
Results: Eleven patients had combined venous lymphatic malformations, three had tufted angiomas, two had a lymphatic malformation, one had a venous malformation, and one had a verrucous venous malformation. All (100%) patients reported some degree of improvement and 50% of patients reported marked improvement in one or more symptoms, most commonly blebs and lymphatic drainage, and bleeding.
Conclusion: Topical sirolimus appears to be a safe and useful non-invasive therapy that is well-tolerated in the treatment of the cutaneous portion of a variety of vascular anomalies.
Monday, August 17, 2020
Pathology:Aggregates of undifferentiated baseloid epithelium, some with central clear cell change and necrosis, associated with a peripheral rim of lymphocytes and scattered plasma cells. In situ hybridization for EBV was negative.
These changes were interpreted as most consistent with a diagnosis of lymphoepithelioma-like carcinoma of the skin.
Thanks to Dr. Lynne Goldberg of Boston University Skin Pathology for the photomicrographs.
|Appearance at re-excision|
ENT Connsult: No evidence of pharyngeal pathology
MRI: Normal with no adenopathy
The patient was referred for Mohs Micrographic Surgery. The tumor was cleared with one stage, but a second stage was done to assure adequacy of excision. (The patient's insurance was not charged for the 2nd stage).
Lymphoepithelioma-like carcinoma of the skin (LELCS) is a rare tumor of unknown etiology, low malignant potential, and microscopic resemblance to undifferentiated nasopharyngeal carcinoma. Clinically (as in this case), it presents as a flesh-colored firm nodule or plaque on the face, scalp, or shoulder of middle-aged to elderly individuals. Histologically, LELCS is composed of islands of enlarged epithelial cells with large vesicular nuclei surrounded and permeated by a dense lymphoplasmacytic infiltrate. LELCS exhibits immunoreactivity with high-molecular-weight cytokeratins and epithelial membrane antigen, indicating the epithelial origin.
Initially, the ENT consult felt that the patient should be referred to a radiotherapist for XRT; but the literature (see ref 3) does not support that. He will be followed up at regular intervals by his dermatologist.
This case presentation highlights the difficulty in treating a rare disorder. No one has enough expertise and one must fall back on the literature that may not be clear. Only about 80 of these tumors have been reported. Consultants may give opinions that are not based on science but on their own belief systems. Where does the buck end? The psychiatrist Michael Balint, described “The Collusion of Anonymity” in his book “The Doctor, His Patient and the Illness.”4 This holds that “the burden of responsibility is much too great, and everyone, including the patient, naturally tries to lighten it by involving someone else, or, if possible, a number of others. This may be described as the process of dilution of responsibility.
If all goes well with this collusion of anonymity all parties concerned feel justifiably proud and gratified. If anything goes wrong nobody is individually responsible.
The collusion of anonymity is one method of lightening the burden of responsibility. Present day practice in medicine is hardly more than the sum of the various specialties.
Nowadays everybody preaches that when a patient is ill the whole person is ill, not only his skin, stomach, his heart or his kidneys. This truth, while constant lip service is paid to it, is unfortunately ignored in most medical practices. A very experienced and disillusioned GP said, "nowadays it is enough for a doctor to know about 20 prescriptions and the address of about 30 consultants."
Wednesday, June 24, 2020
Note: We presented a similar patient two years ago.
Thank you for your thoughts on this patient.
Monday, June 22, 2020
IMPRESSION: These are angiokeratomas of the vulvae. They are different from cherry angiomas. There was one lesion that has been occasionally bleeding although today is quiescent. I could remove that with ED&C when it becomes more active.
Abstract: Angiokeratomas are benign tumors characterized by ectasia of blood vessels in the papillary dermis associated with acanthosis and hyperkeratosis of the epidermis. Dermatological examination of angiokeratomas of Fordyce is characterized by papular keratotic lesions of erythematous-violet color. They are more common in the scrotum, and vulvar involvement is rarely reported. Histopathology is particularly important to distinguish them from other benign and malignant tumors. The article reports the case of a middle-aged black woman with a history of chronic constipation, varicose veins of the lower limbs and cesarean section performed 20 years ago who had had multiple vulvar angiokeratomas for three months.
Friday, June 12, 2020
Monday, May 25, 2020
I made a house call on an 90 year old patient yesterday. He lives with his wife in an idyllic house surrounded by vernal gardens and a wetland preserve.
Postscript: A week after I saw him, he had a PET scan. The next day, he developed profound weakness. He took to his bed. Stopped eating and drinking. He became stuporous and died two days after the scan attended to by his loving wife and a caring nurse. The day after he died, his oncologist called with the PET scan results. His liver was entirely replaced with tumor. He told her that Ketruda was not indicated in such a situation. He lived reasonably well until two weeks before he died.
1. Few people actually benefit from ‘breakthrough’ cancer immunotherapy
By Nathan Gay and Vinay Prasad. March 8, 2017. Stat Topics. [We will try to see if the authors still believe this]
2. Alyson Haslam, Vinay Prasad Estimation of the Percentage of US Patients With Cancer Who Are Eligible for and Respond to Checkpoint Inhibitor Immunotherapy Drugs. JAMA Netw. 2019 May 3;2(5):e192535. Free Full Text at PMC.