HHS Public Access Author manuscript Author Manuscript
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10. Published in final edited form as: Bol Asoc Med P R. 2014 ; 106(4): 43–45.
A very rare neoplasm in an immunocompetent patient Emmanuel O. González-Irizarry, MD1, Javier Ramos Rossy, MD1, Rodolfo Estremera, MD1, Luis A. Figueroa-Jimenez, MD3, Javier Monserrate, MD1, Amy Lee Gonzalez-Marquez, MD3, Luis Negron-Garcia, MD2, Monica Santiago-Casiano, MD2, and William Caceres-Perkins, MD2 1Internal
Medicine Department, VA Caribbean Healthcare System, San Juan, Puerto Rico
Author Manuscript
2Hematology–Medical
Oncology Section, VA Caribbean Healthcare System and San Juan City
Hospital 3Internal
Medicine Department, San Juan City Hospital, San Juan, Puerto Rico
Abstract
Author Manuscript
Kaposi’s sarcoma (KS) is a malignant, slowly progressing, mesenchymal neoplasm characterized by a proliferation of connective tissue and capillaries. It requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated Herpes virus (KSHV) for its development. Four types of KS have been recognized based upon the clinical circumstances in which it develops: classic, endemic, iatrogenic, and AIDS-associated. The course of KS ranges from indolent, with only skin involvement to fulminant with extensive visceral involvement. The exact nature of the disease is not clear. It remains controversial whether the endothelial cells are of vascular origin, lymphatic origin or both. Current data support the notion that KS is a vascular hyperplasia with a tight link to HHV-8 infection. The virus was first identified in KS cells of a patient with AIDS but later, it had been linked convincingly with all 4 types of KS, an association that is necessary, but not sufficient to develop KS. Therefore, other factors are also important. At this point, immunosuppression appears to be the most significant cofactor. Clinical presentation is usually as nodules and red-purple plaques. It occurs most often in older men of Mediterranean or Central/Eastern European ancestry, in whom the lesions usually occur on the distal extremities, particularly the lower legs and feet.
Index words
Author Manuscript
Nodular Kaposi’s sarcoma
Introduction We report a case of a 76-year-old man with a medical history of hypertension, coronary artery disease, and chronic obstructive pulmonary disease that was evaluated at Internal
*
Corresponding author: Emmanuel O. González-Irizarry MD Internal Medicine Department, VA Caribbean Healthcare System, 10 Casia Street San Juan Puerto Rico 00921, [email protected]. Conflict of Interest: The authors declare that there is no conflict of interest.
González-Irizarry et al.
Page 2
Author Manuscript
Medicine Clinics due to recurrent leg cellulitis of 3 months of duration. The leg cellulitis eventually developed a non-healing ulcer and a palpable nodule, unresponsive to local care requiring a node biopsy of the left leg, consistent with a Nodular Kaposi’s sarcoma.
Case History
Author Manuscript
This is a 76-year-old male patient with past medical history of hypertension, unstable angina pectoris, chronic obstructive pulmonary disease, gastroesophageal reflux disease, and colon adenocarcinoma status post resection in year 2012 who presented to his primary care physician with a chief complaint of a left heel lesion at medial aspect of 3 months of evolution (fig. 1, 2). This lesion was an open ulcer of approximately 1 cm of diameter, elevated approximately 3mm, with well-defined borders, draining serous as well as purulent material, and friable (Fig. 2). There was absence of pain or tenderness upon palpation of the area. The patient received oral antibiotics for a few days but the lesions did not improve and later admitted for intravenous antibiotics without significant improvement. He was referred to a dermatologist and pyoderma granuloma was the first diagnostic impression. A skin biopsy was performed and reports the characteristic spindle cells, high vascularity and intracellular hyaline globs and spaces, imparting a pattern that is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma (Fig. 3). No regional lymphadenopathy was present. The patient had a previous negative HIV ELISA tests on 2010 and January 2012. Radiation oncology service was consulted for further management.
Author Manuscript
The patient received 3 sessions of external bean radiotherapy initially which were complicated with lymphedema and disease progression (3 new lesions: 6mm and 2mm on the left lateral foot and a 5mm left medial foot area). He also developed a new lesion at dorsum of right foot. This area also received radiation therapy in two occasions. The clinical course complicated with cellulitis at the left foot with purulent discharge for which treatment with intravenous antibiotics was given with subsequent clinical improvement.
Discussion
Author Manuscript
Kaposi sarcoma (KS) is a neoplasm with abnormal angiogenesis that requires infection with a human herpes virus, HHV-81. This disease is most commonly in elderly men of Mediterranean and Central European descent, but it can also occur in younger individuals. It presents as purplish, reddish blue or dark brown/black skin lesions (which can be macules, nodules, plaques) on the lower extremities. It is a slow-growing tumor, localized and indolent, but it can become disseminated and/or grow rapidly2. The diagnosis is made through biopsy of the lesion(s). Involvement of mucous membranes, lymph nodes, and visceral organs is rare. There are many treatments available but therapeutic choices are often made based upon the experience and medical discipline of the treating clinician and comorbid conditions3. In our patient, diagnosis was made promptly due to the poor response to antibiotics, and the prompt biopsy performed by the dermatology service. Observation rather than specific treatment for patients who have a limited number of asymptomatic lesions that do not impair function is the best approach in these cases4. Many treatments have been used to treat classic KS, although no definitive cure is known. Surgery, formerly recommended, is no longer indicated apart from tissue analysis5. Radiation therapy or
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 3
Author Manuscript
chemotherapy, or both, are preferred mode of treatment5. Radiation therapy is an important treatment, used for many years in classic KS. Lesions of KS are highly radiosensitive, and the treatment is well tolerated and temporarily controls large localized lesions6. For those with limited volume disease causing symptoms (for example, bleeding) or disfigurement; local treatment is recommended. The choice of modality (radiation therapy, excision, cryotherapy, laser ablation) depends on the site and extent of the disease involvement as well as on patient preferences7. Systematic therapy should be considered in the following situations: diffuse symptomatic lesions on multiple body parts that are not easily encompassed in a limited number of radiation fields, diffuse involvement of a large portion of an extremity, bulky disease in a localized area of one limb that cannot be encompassed within a single radiation therapy port, or moderate associated lymphedema beyond what can be controlled with elastic stockings7.
Author Manuscript
Other local therapies that are useful for the management of symptomatic bulky KS lesions or for cosmetic reasons, but they do not prevent the development of new lesions in untreated areas. These therapies include: intralesional chemotherapy, and topical alitretinoin, liposomal anthracyclines and taxanes8. High index of suspicion should be present for those lesions that do not respond to appropriate antibiotic therapy and further assessment is warranted in these cases. Physicians should be aware of this rare entity.
References Author Manuscript Author Manuscript
1. Crosetti, Succo. AIDS-Kaposi Sarcoma and Classic Kaposi Sarcoma: are different ultrasound patterns related to different variants? Journal of Medical Case Reports 2013. 7:293. 2. Jan, Rafi A., Koul, Parvaiz A., et al. Kaposi Sarcoma in a Non HIV Patient. International Journal of Health Sciences. Jul.2008 2(2) 3. Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 isuseful in the diagnosis of Kaposi sarcoma. Mod Pathol. 2004; 17(4):456–460. [PubMed: 14990970] 4. Moore PS, Chang Y. Kaposi’s sarcoma-associated herpes virus immune evasion and tumorigenesis: two sides of the same coin? Annu Rev Microbiol. 2003; 57:609–639. [PubMed: 14527293] 5. Tur E, Brenner S. Treatment of Kaposi’s sarcoma. Arch Dermatol. 1996; 132:327–331. [PubMed: 8607639] 6. Marchell N, Alster TS. Successful treatment of cutaneous Kaposi’s sarcoma by the 585-nm pulsed dye Laser. Dermatol Surg. 1997; 28(10):973–5. 7. Gottlieb JJ, Washenik K, Chachua A, Kien AF. Treatment of classic Kaposi’ sarcoma with liposomal encapsulated doxorubicin. Lancet. 1997; 350(8):1363–4. 8. Phavixay, Laemthong, et al. Case Study: Non AIDS Kaposi’s Sarcoma Leading to Lower Extremities Wounds, Case Presentations and Discussion. Journal of the American College of Clinical Wound Specialists. 2013; 4:13–15. [PubMed: 24527376]
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
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Author Manuscript Author Manuscript Figure 1.
Author Manuscript
Leg Nodules and erythematous/faintly purplish plaques, with associated grayish discoloration of the skin.
Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 5
Author Manuscript Figure 2.
Left foot lesion at medial aspect and interdigits. Lesions are elevated approximately 3mm, with well-defined borders.
Author Manuscript Author Manuscript Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 6
Author Manuscript Figure 3.
Author Manuscript
Surgical Pathology diagnosis: spindle cells, high vascularity and intracellular hyaline globs and spaces, imparting a pattern that is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma.
Author Manuscript Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10. Published in final edited form as: Bol Asoc Med P R. 2014 ; 106(4): 43–45.
A very rare neoplasm in an immunocompetent patient Emmanuel O. González-Irizarry, MD1, Javier Ramos Rossy, MD1, Rodolfo Estremera, MD1, Luis A. Figueroa-Jimenez, MD3, Javier Monserrate, MD1, Amy Lee Gonzalez-Marquez, MD3, Luis Negron-Garcia, MD2, Monica Santiago-Casiano, MD2, and William Caceres-Perkins, MD2 1Internal
Medicine Department, VA Caribbean Healthcare System, San Juan, Puerto Rico
Author Manuscript
2Hematology–Medical
Oncology Section, VA Caribbean Healthcare System and San Juan City
Hospital 3Internal
Medicine Department, San Juan City Hospital, San Juan, Puerto Rico
Abstract
Author Manuscript
Kaposi’s sarcoma (KS) is a malignant, slowly progressing, mesenchymal neoplasm characterized by a proliferation of connective tissue and capillaries. It requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated Herpes virus (KSHV) for its development. Four types of KS have been recognized based upon the clinical circumstances in which it develops: classic, endemic, iatrogenic, and AIDS-associated. The course of KS ranges from indolent, with only skin involvement to fulminant with extensive visceral involvement. The exact nature of the disease is not clear. It remains controversial whether the endothelial cells are of vascular origin, lymphatic origin or both. Current data support the notion that KS is a vascular hyperplasia with a tight link to HHV-8 infection. The virus was first identified in KS cells of a patient with AIDS but later, it had been linked convincingly with all 4 types of KS, an association that is necessary, but not sufficient to develop KS. Therefore, other factors are also important. At this point, immunosuppression appears to be the most significant cofactor. Clinical presentation is usually as nodules and red-purple plaques. It occurs most often in older men of Mediterranean or Central/Eastern European ancestry, in whom the lesions usually occur on the distal extremities, particularly the lower legs and feet.
Index words
Author Manuscript
Nodular Kaposi’s sarcoma
Introduction We report a case of a 76-year-old man with a medical history of hypertension, coronary artery disease, and chronic obstructive pulmonary disease that was evaluated at Internal
*
Corresponding author: Emmanuel O. González-Irizarry MD Internal Medicine Department, VA Caribbean Healthcare System, 10 Casia Street San Juan Puerto Rico 00921, [email protected]. Conflict of Interest: The authors declare that there is no conflict of interest.
González-Irizarry et al.
Page 2
Author Manuscript
Medicine Clinics due to recurrent leg cellulitis of 3 months of duration. The leg cellulitis eventually developed a non-healing ulcer and a palpable nodule, unresponsive to local care requiring a node biopsy of the left leg, consistent with a Nodular Kaposi’s sarcoma.
Case History
Author Manuscript
This is a 76-year-old male patient with past medical history of hypertension, unstable angina pectoris, chronic obstructive pulmonary disease, gastroesophageal reflux disease, and colon adenocarcinoma status post resection in year 2012 who presented to his primary care physician with a chief complaint of a left heel lesion at medial aspect of 3 months of evolution (fig. 1, 2). This lesion was an open ulcer of approximately 1 cm of diameter, elevated approximately 3mm, with well-defined borders, draining serous as well as purulent material, and friable (Fig. 2). There was absence of pain or tenderness upon palpation of the area. The patient received oral antibiotics for a few days but the lesions did not improve and later admitted for intravenous antibiotics without significant improvement. He was referred to a dermatologist and pyoderma granuloma was the first diagnostic impression. A skin biopsy was performed and reports the characteristic spindle cells, high vascularity and intracellular hyaline globs and spaces, imparting a pattern that is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma (Fig. 3). No regional lymphadenopathy was present. The patient had a previous negative HIV ELISA tests on 2010 and January 2012. Radiation oncology service was consulted for further management.
Author Manuscript
The patient received 3 sessions of external bean radiotherapy initially which were complicated with lymphedema and disease progression (3 new lesions: 6mm and 2mm on the left lateral foot and a 5mm left medial foot area). He also developed a new lesion at dorsum of right foot. This area also received radiation therapy in two occasions. The clinical course complicated with cellulitis at the left foot with purulent discharge for which treatment with intravenous antibiotics was given with subsequent clinical improvement.
Discussion
Author Manuscript
Kaposi sarcoma (KS) is a neoplasm with abnormal angiogenesis that requires infection with a human herpes virus, HHV-81. This disease is most commonly in elderly men of Mediterranean and Central European descent, but it can also occur in younger individuals. It presents as purplish, reddish blue or dark brown/black skin lesions (which can be macules, nodules, plaques) on the lower extremities. It is a slow-growing tumor, localized and indolent, but it can become disseminated and/or grow rapidly2. The diagnosis is made through biopsy of the lesion(s). Involvement of mucous membranes, lymph nodes, and visceral organs is rare. There are many treatments available but therapeutic choices are often made based upon the experience and medical discipline of the treating clinician and comorbid conditions3. In our patient, diagnosis was made promptly due to the poor response to antibiotics, and the prompt biopsy performed by the dermatology service. Observation rather than specific treatment for patients who have a limited number of asymptomatic lesions that do not impair function is the best approach in these cases4. Many treatments have been used to treat classic KS, although no definitive cure is known. Surgery, formerly recommended, is no longer indicated apart from tissue analysis5. Radiation therapy or
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 3
Author Manuscript
chemotherapy, or both, are preferred mode of treatment5. Radiation therapy is an important treatment, used for many years in classic KS. Lesions of KS are highly radiosensitive, and the treatment is well tolerated and temporarily controls large localized lesions6. For those with limited volume disease causing symptoms (for example, bleeding) or disfigurement; local treatment is recommended. The choice of modality (radiation therapy, excision, cryotherapy, laser ablation) depends on the site and extent of the disease involvement as well as on patient preferences7. Systematic therapy should be considered in the following situations: diffuse symptomatic lesions on multiple body parts that are not easily encompassed in a limited number of radiation fields, diffuse involvement of a large portion of an extremity, bulky disease in a localized area of one limb that cannot be encompassed within a single radiation therapy port, or moderate associated lymphedema beyond what can be controlled with elastic stockings7.
Author Manuscript
Other local therapies that are useful for the management of symptomatic bulky KS lesions or for cosmetic reasons, but they do not prevent the development of new lesions in untreated areas. These therapies include: intralesional chemotherapy, and topical alitretinoin, liposomal anthracyclines and taxanes8. High index of suspicion should be present for those lesions that do not respond to appropriate antibiotic therapy and further assessment is warranted in these cases. Physicians should be aware of this rare entity.
References Author Manuscript Author Manuscript
1. Crosetti, Succo. AIDS-Kaposi Sarcoma and Classic Kaposi Sarcoma: are different ultrasound patterns related to different variants? Journal of Medical Case Reports 2013. 7:293. 2. Jan, Rafi A., Koul, Parvaiz A., et al. Kaposi Sarcoma in a Non HIV Patient. International Journal of Health Sciences. Jul.2008 2(2) 3. Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 isuseful in the diagnosis of Kaposi sarcoma. Mod Pathol. 2004; 17(4):456–460. [PubMed: 14990970] 4. Moore PS, Chang Y. Kaposi’s sarcoma-associated herpes virus immune evasion and tumorigenesis: two sides of the same coin? Annu Rev Microbiol. 2003; 57:609–639. [PubMed: 14527293] 5. Tur E, Brenner S. Treatment of Kaposi’s sarcoma. Arch Dermatol. 1996; 132:327–331. [PubMed: 8607639] 6. Marchell N, Alster TS. Successful treatment of cutaneous Kaposi’s sarcoma by the 585-nm pulsed dye Laser. Dermatol Surg. 1997; 28(10):973–5. 7. Gottlieb JJ, Washenik K, Chachua A, Kien AF. Treatment of classic Kaposi’ sarcoma with liposomal encapsulated doxorubicin. Lancet. 1997; 350(8):1363–4. 8. Phavixay, Laemthong, et al. Case Study: Non AIDS Kaposi’s Sarcoma Leading to Lower Extremities Wounds, Case Presentations and Discussion. Journal of the American College of Clinical Wound Specialists. 2013; 4:13–15. [PubMed: 24527376]
Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 4
Author Manuscript Author Manuscript Figure 1.
Author Manuscript
Leg Nodules and erythematous/faintly purplish plaques, with associated grayish discoloration of the skin.
Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 5
Author Manuscript Figure 2.
Left foot lesion at medial aspect and interdigits. Lesions are elevated approximately 3mm, with well-defined borders.
Author Manuscript Author Manuscript Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
González-Irizarry et al.
Page 6
Author Manuscript Figure 3.
Author Manuscript
Surgical Pathology diagnosis: spindle cells, high vascularity and intracellular hyaline globs and spaces, imparting a pattern that is strikingly reminiscent of a cavernous hemangioma consistent with nodular Kaposi’s sarcoma.
Author Manuscript Author Manuscript Bol Asoc Med P R. Author manuscript; available in PMC 2017 April 10.
